Polycythemia code icd 10. Polycythemia. Preparations for normalizing the state of blood aggregation

Code D45 will continue to be used, although it is in the chapter on neoplasms of undetermined or unknown nature. Modification of its classification is reserved for revision of the ICD.

Alkylating agent-associated myelodysplastic syndrome

Myelodysplastic syndrome associated with epipodophyllotoxin

Myelodysplastic syndrome associated with NOS therapy

Excludes: drug-induced aplastic anemia (D61.1)

In Russia, the International Classification of Diseases, 10th revision (ICD-10) has been adopted as a single normative document for recording morbidity, reasons for the population's visits to medical institutions of all departments, and causes of death.

ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. No. 170

The release of a new revision (ICD-11) is planned by WHO in 2017-2018.

With changes and additions from WHO.

Processing and translation of changes © mkb-10.com

Secondary polycythemia

Definition and general information [edit]

Synonyms: secondary erythrocytosis

Secondary polycythemia is a condition of increased absolute erythrocyte mass caused by increased stimulation of erythrocyte production in the presence of a normal erythroid lineage, which may be congenital or acquired.

Etiology and pathogenesis[edit]

Secondary polycythemia may be congenital and caused by defects in the oxygen sensing pathway due to autosomal recessive mutations in the VHL (3p26-p25), EGLN1 (1q42-q43) and EPAS1 (2p21-p16) genes, leading to increased erythropoietin production in the setting of hypoxia; or other autosomal dominant birth defects, including high-oxygen-affinity hemoglobin and bisphosphoglycerate mutase deficiency, which leads to tissue hypoxia and secondary erythrocytosis.

Secondary polycythemia may also be caused by an increase in the amount of erythropoietin due to tissue hypoxia, which may be central as a result of diseases of the lungs and heart or exposure to high altitude, or local, such as renal hypoxia due to renal artery stenosis.

Erythropoietin production may be abnormal due to erythropoietin-secreting tumors—renal cancer, hepatocellular carcinoma, cerebellar hemangioblastoma, meningioma, and parathyroid carcinoma/adenoma. In addition, erythropoietin can be administered intentionally as a doping agent to athletes.

Clinical manifestations[edit]

Clinical features vary depending on the etiology of polycythemia, but typically symptoms may include plethora, flushed complexion, headache, and tinnitus. The congenital form may be accompanied by thrombophlebitis of the superficial or deep veins, may be associated with specific symptoms, as in the case of Chuvash familial erythrocytosis, or the course of the disease may be indolent.

Patients with a specific subtype of congenital secondary polycythemia, known as Chuvash erythrocytosis, have lower systolic or diastolic blood pressure, varicose veins, vertebral body hemangiomas, as well as cerebrovascular complications and mesenteric thrombosis.

The acquired form of secondary polycythemia can manifest as cyanosis, hypertension, drumsticks on the legs and arms, and drowsiness.

Secondary polycythemia: Diagnosis [edit]

Diagnosis is based on detection of an increase in the total number of red blood cells and normal or elevated serum erythropoietin levels. Secondary causes of erythrocytosis must be diagnosed individually and will require comprehensive evaluation.

Differential diagnosis[edit]

The differential diagnosis includes polycythemia vera and primary familial polycythemia, which can be excluded by the presence of low levels of erythropoietin and mutations in the JAK2 gene (9p24) in polycythemia.

Secondary polycythemia: Treatment[edit]

Phlebotomy or venesection may be beneficial, especially in patients at increased risk of thrombosis. A target hematocrit (Hct) of 50% may be most optimal. Low dose aspirin may be beneficial. In acquired cases of secondary polycythemia, patient management is based on treatment of the underlying condition. Forecast

The prognosis mainly depends on the concomitant disease in acquired forms of secondary erythrocytosis and the severity of thrombotic complications in hereditary forms, such as Chuvash erythrocytosis.

Prevention[edit]

Other [edit]

Synonyms: stress erythrocytosis, stress polycythemia, stress polycythemia

Heisbock syndrome is characterized by secondary polycythemia and occurs mainly in men on a high-calorie diet.

The prevalence of Heisbock syndrome is unknown.

The clinical picture of Heisbock syndrome includes mild obesity, hypertension, and decreased plasma volume with a relative increase in hematocrit, increased blood viscosity, and elevated serum cholesterol, triglycerides, and uric acid. The decrease in plasma volume appears to be associated with an increase in diastolic blood pressure.

The prognosis is worsened by the development of cardiovascular complications.

Polycythemia

ICD-10 code

Titles

Description

Symptoms

There are several stages in the clinical course:

*initial, or asymptomatic, stage, usually lasting 5 years, with minimal clinical manifestations;

*stage IIA - erythraemic advanced stage, without myeloid metaplasia of the spleen, its duration can reach years;

*stage IIB - erythremic advanced stage, with myeloid metaplasia of the spleen;.

*stage III - stage of post-erythraemic myeloid metaplasia (anemic stage) with or without myelofibrosis; possible outcome in acute leukemia, chronic myeloid leukemia.

However, given the usual onset of the disease in elderly and old people, not all patients go through all three stages.

In the anamnesis of many patients, long before the time of diagnosis, there are indications of bleeding after tooth extraction, skin itching associated with water procedures, “good”, somewhat elevated red blood counts, and a duodenal ulcer. An increase in the mass of circulating erythrocytes leads to an increase in blood viscosity, stasis in the microvasculature, and an increase in peripheral vascular resistance, therefore the skin of the face, ears, tip of the nose, distal parts of the fingers and visible mucous membranes have a red-cyanotic color of varying degrees. Increased viscosity explains the high frequency of vascular, mainly cerebral, complaints: headache, dizziness, insomnia, a feeling of heaviness in the head, blurred vision, tinnitus. Possible epileptiform seizures, depression, paralysis. Patients complain of progressive memory loss. In the initial stage of the disease, arterial hypertension is found in% of patients. Cellular hypercatabolism and partially ineffective erythropoiesis cause increased endogenous synthesis of uric acid and impaired urate metabolism. Clinical manifestations of urate (uric acid) diathesis are renal colic, gout, complicating the course of stages IIB and III. Visceral complications include ulcers of the stomach and duodenum; their frequency, according to various authors, ranges from 10 to 17%.

Vascular complications pose the greatest danger to patients with polycythemia. A unique feature of this disease is the simultaneous tendency to both thrombosis and bleeding. Microcirculatory disorders as a consequence of thrombophilia are manifested by erythromelalgia - severe redness and swelling of the distal parts of the fingers and toes, accompanied by burning pain. Persistent erythromelalgia may be a harbinger of thrombosis of a larger vessel with the development of necrosis of the fingers, feet, and legs. Thrombosis of coronary vessels is observed in 7-10% of patients. The development of thrombosis is facilitated by a number of factors: age over 60 years, a history of vascular thrombosis, arterial hypertension, atherosclerosis of any localization, blood exfusion or plateletpheresis performed without anticoagulant or disaggregant therapy. Thrombotic complications, in particular myocardial infarction, ischemic stroke and pulmonary embolism, are the most common cause of death in these patients.

Hemorrhagic syndrome is manifested by spontaneous bleeding of the gums, nosebleeds, ecchymoses, characteristic of disorders of the platelet-vascular component of hemostasis.

Pathogenesis

The spleen enlarges in stage IIA, the reason for this is the increased deposition and sequestration of blood cells. In stage IIB, splenomegaly is caused by progressive myeloid metaplasia. It is accompanied by a left shift in the leukocyte formula and erythrokaryocytosis. Liver enlargement often accompanies splenomegaly. Both stages are characterized by liver fibrosis. The course of the post-erythremic stage is variable. In some patients it is completely benign, the spleen and liver enlarge slowly, and red blood counts remain within normal limits for a long time. At the same time, rapid progression of splenomegaly, an increase in anemia, an increase in leukocytosis and the development of blast transformation are also possible. Acute leukemia can develop both in the erythraemic stage and in the stage of posterythremic myeloid metaplasia.

Causes

The main causes of secondary erythrocytosis include tissue hypoxia, both congenital and acquired, and changes in the content of endogenous erythropoietin.

Causes of secondary erythrocytosis:

1,high affinity of hemoglobin for oxygen;.

2,low level of 2,3-diphosphoglycerate;.

3,autonomous production of erythropoietin.

1, arterial hypoxemia of physiological and pathological nature:

“blue” heart defects;.

Chronic pulmonary diseases;

Adaptation to high mountain conditions.

Diffuse diseases of the renal parenchyma;

Renal artery stenosis.

Treatment

Planned therapy. Modern therapy for erythremia consists of the use of blood exfusions, cytostatic drugs, the use of radioactive phosphorus, and interferon.

Bloodletting, which gives a quick clinical effect, can be an independent method of treatment or complement cytostatic therapy. In the initial stage, which occurs with an increase in the content of red blood cells, 2-3 bloodlettings of 500 ml are used every 3-5 days, followed by the introduction of adequate amounts of rheopolyglucin or saline. In patients with cardiovascular diseases, no more than 350 ml of blood is removed per procedure, exfusions no more than once a week. Phlebotomy does not control white blood cell and platelet counts, sometimes causing reactive thrombocytosis. Usually, skin itching, erythromelalgia, gastric and duodenal ulcers, and uric acid diathesis are not eliminated by bloodletting. They can be replaced by erythrocytapheresis with replacement of the volume of removed red blood cells with saline solution and rheopolyglucin. The procedure is well tolerated by patients and causes normalization of red blood counts for a period of 8 to 12 months.

Cytostatic therapy is aimed at suppressing the increased proliferative activity of the bone marrow; its effectiveness should be assessed after 3 months. After the end of treatment, although the decrease in the number of leukocytes and platelets occurs much earlier.

The indication for cytostatic therapy is erythremia occurring with leukocytosis, thrombocytosis and splenomegaly, skin itching, visceral and vascular complications; insufficient effect from previous bloodlettings, their poor tolerance.

Contraindications to cytostatic therapy are childhood and adolescence of patients, refractoriness to treatment at previous stages, overly active cytostatic therapy is also contraindicated due to the risk of hematopoietic depression.

The following drugs are used to treat erythremia:

*alkylating agents - myelosan, alkeran, cyclophosphamide.

*hydroxyurea, which is the drug of choice, in dosemg/kg/day. After a decrease in the number of leukocytes and platelets, the daily dose is reduced to 15 mg/kg for 2-4 weeks. , subsequently a maintenance dose of 500 mg/day is prescribed.

A new direction in the treatment of polycythemia is the use of interferon drugs, aimed at reducing myeloproliferation, platelet count and vascular complications. Time of onset of therapeutic effect: months. Normalization of all blood parameters is assessed as an optimal effect, a reduction in the need for erythrocyte exfusion by 50% is assessed as incomplete. During the period of achieving the effect, it is recommended to prescribe 9 million units/day 3 times a week, with a transition to a maintenance dose selected individually. Treatment is usually well tolerated and lasts for many years. One of the undoubted advantages of the drug is the absence of leukemia.

To improve the quality of life, patients are given symptomatic therapy:

*uric acid diathesis (with clinical manifestations of urolithiasis, gout) requires constant intake of allopurinol (miluritis) in a daily dose of 200 mg to 1 g;

*erythromelalgia is an indication for prescribing 500 mg of aspirin or 250 mg of methindol; for severe erythromelalgia, additional heparin is indicated;

*for vascular thrombosis, disaggregants are prescribed; in case of hypercoagulation, according to coagulogram data, heparin should be prescribed in a single dose of 5000 units 2-3 times a day. The dose of heparin is determined by monitoring the coagulation system. Acetylsalicylic acid is most effective in the prevention of thrombophilic complications, but its use threatens hemorrhagic dose-dependent complications. The basic prophylactic dose of aspirin is 40 mg of the drug per day;

*skin itching is somewhat relieved by antihistamines; interferon has a significant, but slower (not earlier than 2 months) effect.

POLYCYTHEMIA TRUE

Polycythemia vera or Vaquez's disease is a myeloproliferative disease with the formation of a tumor bone marrow clone of progenitor cells capable of differentiating into mature erythrocytes, granulocytes, and platelets.

ICD10:D45 – Polycythemia vera.

Latent viral infection may be important in the etiology of polycythemia vera.

As a result of a virus-induced mutation, an additional, tumor-producing clone of progenitor cells appears in the bone marrow. Like the normal one, the tumor clone retains the ability to form erythrocyte, granulocytic, and megakaryocytic hematopoietic lines. These lines reach final differentiation to mature erythrocytes, granulocytes, and platelets. Although blood cells (both normal and tumor generation) are intensively destroyed by fixed macrophages of the spleen, as evidenced by the increased level of uric acid and bilirubin in the blood, three-line polycythemia is formed: erythrocytosis, granulocytosis, thrombocytosis. Due to the “failure” to fully fulfill its function of eliminating excess formed blood elements from the blood circulation, the spleen enlarges compensatoryly. Erythrocytosis, through a feedback mechanism, regulatory suppresses the production of erythropoietin. The tumor clone of hematopoiesis, insensitive to erythropoietin, expands its bridgehead, metastasizing to the spleen, liver and other organs. Apparently, in order to eliminate the uncontrolled tumor line of hematopoiesis, the body turns on the immune mechanisms of total suppression of myelopoiesis. As a result, polycythemia vera develops into another disease - myelofibrosis with devastation of the bone marrow, the formation of aplastic anemia. Additional mutations as a result of viral passages, evasion of hematopoietic cells from autoimmune myelotoxic effects, intoxication with cytostatics and radioactive phosphorus can cause the appearance of uncontrolled tumor clones of hematopoietic cells with the formation of acute leukemia.

In the pathogenesis of the advanced stage of the disease, the abnormally high content of erythrocytes in the peripheral blood is of leading importance. This increases its viscosity, leading to hemocirculation disorders, excessive plethora of organs and tissues with a compensatory (viscous blood needs to be pushed through) increase in blood pressure. Various pathological reactions occur due to the high content of granulocytes and platelets in the blood: thrombosis, hemorrhagic syndrome.

The disease begins unnoticed and progresses slowly.

In the advanced phase, due to erythrocytosis, patients begin to experience dizziness, headache, tinnitus, sensations of fullness and hot flashes to the head, visual disturbances in the form of double vision, red spots in the eyes, fainting, a tendency to convulsions, itchy skin. Progressive bone marrow hyperplasia causes bursting pain in the bones.

Many people are bothered by pain in the heart area, in the epigastric region, in the left hypochondrium in the projection of the enlarged spleen.

A characteristic symptom is erythromelalgia: burning, unbearable pain in the fingertips, which can be temporarily relieved by taking aspirin. Necrosis may occur on the distal phalanges of the fingers.

Worried about nosebleeds and stomach bleeding.

Thrombosis of cerebral vessels with characteristic focal neurological symptoms may occur. Coronary artery thrombosis, not associated with atherosclerosis, is the main cause of myocardial infarction in patients with polycythemia vera.

An objective examination reveals plethora (plethora): purplish-cyanotic complexion, brightly colored lips, pronounced hyperemia of the conjunctiva (“rabbit eyes”), bright red tongue and soft palate with a distinct border of transition to the hard palate. The skin of the trunk and limbs is pink, the saphenous veins are dilated.

Skin of the lower extremities with areas of pigmentation caused by disturbances in the blood flow of viscous blood in small venous vessels.

Splenomegaly is a typical sign of polycythemia vera. It is often combined with hepatomegaly.

The boundaries of the heart are expanded. Blood pressure is increased. Ulcers of the stomach and duodenum may form. Against the background of hyperuricemia, caused by intensive breakdown of granulocytes in the spleen, symptoms of secondary gout and urolithiasis appear.

Due to nosebleeds and as a result of bloodletting, the patient may develop sideropenic syndrome.

The clinical course of the disease is divided into three stages:

1. The initial stage lasts about 5 years. It is characterized by moderate erythrocytosis, small plethora, absence of splenomegaly, and rare vascular and thrombotic complications. Three-line hyperplasia of the bone marrow is detected.

2. Advanced erythremic stage lasting more than 10 years, which is divided into two substages.

a. Without myeloid metaplasia of the spleen. It is characterized by severe plethora, erythromelalgia, splenomegaly, panmyelosis - severe erythromyeloid and megakaryocytic hyperplasia of the bone marrow with replacement of fatty marrow with red. Thrombotic complications often occur in the form of heart attacks, strokes, and necrosis of the fingertips.

b. With myeloid metaplasia of the spleen. It manifests itself as severe splenomegaly, hepatomegaly, moderately severe plethora, panmyelosis, bleeding, and thrombotic complications.

3. Terminal anemic stage. Corresponds to the formation of myelofibrosis. It manifests itself as aplastic anemia with pancytopenia, severe splenomegaly, and hepatomegaly. At this stage, the disease can transform into chronic myeloid leukemia or acute leukemia. Especially in cases where radioactive phosphorus and cytostatics are used for treatment.

General blood test: erythrocytosis above 5.7x10 9 /l, hemoglobin more than 177 g/l. Thrombocytosis. Neutrophilic leukocytosis with a shift to the left to single metamyelocytes and myelocytes. ESR is reduced to 0.5-1 mm/hour.

Blood viscosity is 5-8 times higher than normal.

Hematocrit: above 52%.

Biochemical blood test: increased uric acid, moderate increase in bilirubin levels.

Sternal puncture: pronounced hyperplasia of all three lines of myelopoiesis - erythrocyte, granulocytic, megakaryocyte, with replacement of the fatty marrow with red. In the terminal stage there are signs of myelofibrosis.

Polycythemia vera

Polycythemia vera (erythremia, Vaquez disease or primary polycythemia) is a progressive malignant disease belonging to the group of leukemias, which is associated with hyperplasia of the cellular elements of the bone marrow (myeloproliferation). The pathological process primarily affects the erythroblastic germ, so an excess number of red blood cells is detected in the blood. An increase in the number of neutrophilic leukocytes and platelets is also observed.

An increased number of red blood cells increases blood viscosity, increases its mass, causes a slowdown in blood flow in the vessels and the formation of blood clots. As a result, patients develop impaired blood supply and hypoxia.

General information

Polycythemia vera was first described in 1892 by the French physician and cardiologist Vaquez. Vaquez suggested that the hepatosplenomegaly and erythrocytosis detected in his patient arose as a result of increased proliferation of hematopoietic cells, and identified erythremia as a separate nosological form.

In 1903, W. Osler used the term “Vaquez disease” to describe patients with splenomegaly (enlarged spleen) and severe erythrocytosis and gave a detailed description of the disease.

Turk (W. Turk) in 1902-1904 suggested that in this disease the disorder of hematopoiesis is hyperplastic in nature, and called the disease erythremia by analogy with leukemia.

The clonal neoplastic nature of myeloproliferation, which is observed in polycythemia, was proven in 1980 by P. J. Fialkov. He discovered one type of enzyme, glucose-6-phosphate dehydrogenase, in red blood cells, granulocytes and platelets. In addition, both types of this enzyme were detected in the lymphocytes of two patients heterozygous for this enzyme. Thanks to Fialkov's research, it became clear that the target of the neoplastic process is the precursor cell of myelopoiesis.

In 1980, a number of researchers managed to separate the neoplastic clone from normal cells. It has been experimentally proven that polycythemia produces a population of erythroid committed precursors that are pathologically highly sensitive to even small amounts of erythropoietin (a kidney hormone). According to scientists, this contributes to increased formation of red blood cells in polycythemia vera.

In 1981, L. D. Sidorova and co-authors conducted studies that made it possible to detect qualitative and quantitative changes in the platelet component of hemostasis, which play a major role in the development of hemorrhagic and thrombotic complications in polycythemia.

Polycythemia vera is detected mainly in older people, but can be observed in young people and children. In young people, the disease is more severe. The average age of patients varies from 50 to 70 years. The average age of those who become ill for the first time is gradually increasing (in 1912 it was 44 years, and in 1964 - 60 years). The number of patients under 40 years of age is about 5%, and erythremia in children and patients under 20 years of age is detected in 0.1% of all cases of the disease.

Erythremia is slightly less common in women than in men (1: 1.2-1.5).

It is the most common disease in the group of chronic myeloproliferative diseases. It is quite rare - according to various sources, from 5 to 29 cases in the population.

There is isolated data on the influence of racial factors (above the average among Jews and below the average among representatives of the Negroid race), but at the moment this assumption has not been confirmed.

Forms

Polycythemia vera is divided into:

Primary (not a consequence of other diseases).
Secondary. It can be triggered by chronic lung disease, hydronephrosis, the presence of tumors (uterine fibroids, etc.), the presence of abnormal hemoglobins and other factors associated with tissue hypoxia.

An absolute increase in erythrocyte mass is observed in all patients, but only in 2/3 the number of leukocytes and platelets also increases.

Reasons for development

The causes of polycythemia vera have not been definitively established. Currently, there is no single theory that would explain the occurrence of hemoblastoses (blood tumors), to which this disease belongs.

Based on epidemiological observations, a theory was put forward about the connection of erythremia with the transformation of stem cells, which occurs under the influence of gene mutations.

It has been established that most patients have a mutation in the enzyme Janus kinase-tyrosine kinase, synthesized in the liver, which is involved in the transcription of certain genes by phosphorylating many tyrosines in the cytoplasmic part of the receptors.

The most common mutation, discovered in 2005, is in exon 14 JAK2V617F (detected in 96% of all cases of the disease). In 2% of cases, the mutation affects exon 12 of the JAK2 gene.

Patients with polycythemia vera also have:

In some cases, mutations in the thrombopoietin receptor gene MPL. These mutations are of secondary origin and are not strictly specific for this disease. They are detected in older people (mainly women) with low levels of hemoglobin and platelets.
Loss of function of the LNK gene protein SH2B3, which reduces the activity of the JAK2 gene.

Elderly patients with a high JAK2V617F allelic load are characterized by elevated hemoglobin levels, leukocytosis and thrombocytopenia.

With a mutation of the JAK2 gene in exon 12, erythremia is accompanied by a subnormal serum level of the hormone erythropoietin. Patients with this mutation are younger.

In polycythemia vera, mutations of TET2, IDH, ASXL1, DNMT3A, etc. are also often detected, but their pathogenetic significance has not yet been studied.

There were no differences in survival of patients with different types of mutations.

As a result of molecular genetic disorders, the JAK-STAT signaling pathway is activated, which is manifested by proliferation (cell production) of the myeloid lineage. At the same time, proliferation and an increase in the number of red blood cells in the peripheral blood increase (an increase in the number of leukocytes and platelets is also possible).

The identified mutations are inherited in an autosomal recessive manner.

There is also a hypothesis according to which the cause of erythremia may be viruses (15 types of such viruses have been identified), which, in the presence of predisposing factors and weakened immunity, penetrate into immature bone marrow cells or lymph nodes. Cells affected by the virus begin to actively divide instead of maturing, thus starting the pathological process.

Factors that provoke the disease include:

X-ray irradiation, ionizing radiation;
paints, varnishes and other toxic substances that penetrate the human body;
long-term use of certain medications for medicinal purposes (gold salts for rheumatoid arthritis, etc.);
viral and intestinal infections, tuberculosis;
surgical interventions;
stressful situations.

Secondary erythremia develops under the influence of favorable factors when:

high innate affinity of hemoglobin for oxygen;
low levels of 2,3-diphosphoglycerate;
autonomous production of erythropoietin;
arterial hypoxemia of a physiological and pathological nature (“blue” heart defects, smoking, adaptation to high altitude conditions and chronic lung diseases);
kidney diseases (cystic lesions, hydronephrosis, renal artery stenosis and diffuse diseases of the renal parenchyma);
the presence of tumors (possibly influenced by bronchial carcinoma, cerebellar hemangioblastoma, uterine fibroids);
endocrine diseases associated with adrenal tumors;
liver diseases (cirrhosis, hepatitis, hepatoma, Budd-Chiari syndrome);
tuberculosis.

Pathogenesis

The pathogenesis of polycythemia vera is associated with a disruption of the process of hematopoiesis (hematopoiesis) at the level of the progenitor cell. Hematopoiesis acquires the unlimited proliferation of progenitor cells characteristic of a tumor, the descendants of which form a specialized phenotype in all hematopoietic lineages.

Polycythemia vera is characterized by the formation of erythroid colonies in the absence of exogenous erythropoietin (the appearance of endogenous erythropoietin-independent colonies is a sign that distinguishes erythremia from secondary erythrocytosis).

The formation of erythroid colonies indicates a disruption in the implementation of regulatory signals that the myeloid cell receives from the external environment.

The basis of the pathogenesis of polycythemia vera is defects in genes encoding proteins that are responsible for maintaining myelopoiesis within the normal range.

A decrease in oxygen concentration in the blood causes a reaction in the interstitial cells of the kidneys that synthesize erythropoietin. The process occurring in interstitial cells concerns the work of many genes. The main regulation of this process is carried out by factor-1 (HIF-1), which is a heterodimeric protein consisting of two subunits (HIF-1alpha and HIF-1beta).

If the oxygen concentration in the blood is within normal limits, proline residues (the heterocyclic amino acid of the freely existing HIF-1 molecule) are hydroxylated under the influence of the regulatory enzyme PHD2 (molecular oxygen sensor). Thanks to hydroxylation, the HIF-1 subunit acquires the ability to bind to the VHL protein, which provides tumor prevention.

The VHL protein forms a complex with a number of E3 ubiquitin ligase proteins, which, after forming covalent bonds with other proteins, are sent to the proteasome and destroyed there.

During hypoxia, hydroxylation of the HIF-1 molecule does not occur; the subunits of this protein combine and form the heterodimeric HIF-1 protein, which travels from the cytoplasm to the nucleus. Once in the nucleus, the protein binds to special DNA sequences in the promoter regions of genes (the conversion of genes into protein or RNA is induced by hypoxia). As a result of these transformations, erythropoietin is released into the bloodstream by the interstitial cells of the kidneys.

By myelopoiesis precursor cells, the genetic program embedded in them is carried out as a result of the stimulating effect of cytokines (these small peptide control (signal) molecules bind to the corresponding receptors on the surface of the precursor cells).

When erythropoietin binds to the erythropoietin receptor EPO-R, dimerization of this receptor occurs, which activates the Jak2 kinase associated with the intracellular domains of EPO-R.

Jak2 kinase is responsible for signal transmission from erythropoietin, thrombopoietin and G-CSF (granulocyte colony-stimulating factor).

Due to the activation of Jak2-kinase, phospholation of a number of cytoplasmic target proteins occurs, which includes adapter proteins of the STAT family.

Erythremia was detected in 30% of patients with constitutive activation of the STAT3 gene.

Also, with erythremia, in some cases, a reduced level of expression of the thrombopoietin receptor MPL is detected, which is compensatory in nature. The reduction in MPL expression is secondary and is caused by a genetic defect responsible for the development of polycythemia vera.

A decrease in degradation and an increase in the level of the HIF-1 factor is caused by defects in the VHL gene (for example, representatives of the population of Chuvashia are characterized by a homozygous mutation 598C>T of this gene).

Polycythemia vera can be caused by abnormalities of chromosome 9, but the most common is a deletion of the long arm of chromosome 20.

In 2005, a point mutation in exon 14 of the Jak2 kinase gene (mutation JAK2V617F) was identified, which causes the replacement of the amino acid valine with phenylalanine in the pseudokinase domain JH2 of the JAK2 protein at position 617.

The JAK2V617F mutation in hematopoietic precursor cells in erythremia is presented in a homozygous form (the formation of the homozygous form is affected by mitotic recombination and duplication of the mutant allele).

When JAK2V617F and STAT5 are active, the level of reactive oxygen species increases, resulting in a transition of the cell cycle from the G1 to S phase. The adapter protein STAT5 and reactive oxygen species transmit a regulatory signal from JAK2V617F to the cyclin D2 and p27kip genes, which causes an accelerated transition of the cell cycle from phase G1 to S. As a result, the proliferation of erythroid cells that carry a mutant form of the JAK2 gene increases.

In JAK2V617F-positive patients, this mutation is detected in myeloid cells, B- and T-lymphocytes and natural killer cells, which proves the proliferative advantage of defective cells compared to the norm.

Polycythemia vera in most cases is characterized by a fairly low ratio of mutant to normal allele in mature myeloid cells and early precursors. In the presence of clonal dominance, patients have a more severe clinical picture compared to patients without this defect.

Symptoms

Symptoms of polycythemia vera are associated with excess production of red blood cells, which increase blood viscosity. In most patients, the level of platelets, which cause vascular thrombosis, also increases.

The disease develops very slowly and is asymptomatic at the initial stage.

At later stages, polycythemia vera manifests itself:

plethoric syndrome, which is associated with increased blood supply to organs;
myeloproliferative syndrome, which occurs with increased production of red blood cells, platelets and leukocytes.

Plethoric syndrome is accompanied by:

Headaches.
Feeling of heaviness in the head;
Dizziness.
Attacks of pressing, squeezing pain behind the sternum, which occurs during physical activity.
Erythrocyanosis (redness of the skin to a cherry tint and a bluish tint of the tongue and lips).
Redness of the eyes, which occurs as a result of dilation of blood vessels in them.
A feeling of heaviness in the upper abdomen (left), which occurs as a result of an enlarged spleen.
Skin itching, which is observed in 40% of patients (a specific sign of the disease). It intensifies after water procedures and occurs as a result of irritation by the breakdown products of red blood cells of the nerve endings.
An increase in blood pressure, which decreases well with bloodletting and decreases slightly with standard treatment.
Erythromelalgia (sharp, burning pain in the fingertips that is relieved by taking blood thinners, or painful swelling and redness of the foot or lower third of the leg).

Myeloproliferative syndrome manifests itself:

soreness in flat bones and joint pain;
a feeling of heaviness in the right upper abdomen as a result of an enlarged liver;
general weakness and increased fatigue;
increase in body temperature.

Varicose veins are also observed, especially noticeable in the neck area, Cooperman's sign (change in color of the soft palate with normal coloration of the hard palate), duodenal ulcer and, in some cases, stomach, bleeding of the gums and esophagus, and increased uric acid levels. The development of heart failure and cardiosclerosis is possible.

Stages of the disease

Polycythemia vera is characterized by three stages of development:

Initial, stage I, which lasts about 5 years (a longer period is possible). It is characterized by moderate manifestations of plethoric syndrome, the size of the spleen does not exceed the norm. A general blood test reveals a moderate increase in the number of red blood cells; increased formation of red blood cells is observed in the bone marrow (an increase in the number of all blood cells, with the exception of lymphocytes, is also possible). At this stage, complications practically do not arise.
The second stage, which can be polycythemic (II A) and polycythemic with myeloid metaplasia of the spleen (II B). Form II A, lasting from 5 to 15 years, is accompanied by severe plethoric syndrome, enlargement of the liver and spleen, the presence of thrombosis, and bleeding. Tumor growth in the spleen is not detected. Possible iron deficiency due to frequent bleeding. A general blood test reveals an increase in the number of red blood cells, platelets and leukocytes. Scar changes are observed in the bone marrow. Form II B is characterized by progressive enlargement of the liver and spleen, the presence of tumor growth in the spleen, thrombosis, general exhaustion, and bleeding. A complete blood count can detect an increase in the number of all blood cells, with the exception of lymphocytes. Red blood cells take on different sizes and shapes, and immature blood cells appear. Scar changes in the bone marrow gradually increase.
Anemic, stage III, which develops a year after the onset of the disease and is accompanied by a pronounced enlargement of the liver and spleen, extensive cicatricial changes in the bone marrow, circulatory disorders, a decrease in the number of red blood cells, platelets and leukocytes. Transformation into acute or chronic leukemia is possible.

Diagnostics

Erythremia is diagnosed based on:

Analysis of complaints, medical history and family history, during which the doctor clarifies when the symptoms of the disease appeared, what chronic diseases the patient has, whether there was contact with toxic substances, etc.
Data from a physical examination, which pays attention to the color of the skin. During palpation and with the help of percussion (tapping), the size of the liver and spleen is determined, pulse and blood pressure are also measured (may be elevated).
A blood test that determines the number of red blood cells (the norm is 4.0-5.5x109 g/l), leukocytes (can be normal, increased or decreased), platelets (at the initial stage does not deviate from the norm, then an increase in the level is observed, and then a decrease ), hemoglobin level, color indicator (usually the norm is 0.86-1.05). ESR (erythrocyte sedimentation rate) is reduced in most cases.
Urinalysis, which allows you to identify concomitant diseases or the presence of renal bleeding.
A biochemical blood test that reveals the increased level of uric acid characteristic of many cases of the disease. To identify organ damage accompanying the disease, the level of cholesterol, glucose, etc. is also determined.
Data from a bone marrow study, which is performed using a puncture in the sternum and reveals increased production of red blood cells, platelets and leukocytes, as well as the formation of scar tissue in the bone marrow.
Trepanobiopsy data, which most fully reflect the condition of the bone marrow. For examination, using a special trephine device, a column of bone marrow is taken from the wing of the ilium along with the bone and periosteum.

A coagulogram, iron metabolism studies are also performed, and the level of erythropoietin in the blood serum is determined.

Since chronic erythremia is accompanied by an enlargement of the liver and spleen, an ultrasound of the internal organs is performed. Ultrasound also detects the presence of hemorrhages.

To assess the extent of the tumor process, SCT (spiral computed tomography) and MRI (magnetic resonance imaging) are performed.

To identify genetic abnormalities, a molecular genetic study of peripheral blood is performed.

Treatment

The goals of treatment for polycythemia vera are:

prevention and treatment of thrombohemorrhagic complications;
elimination of symptoms of the disease;
reducing the risk of complications and development of acute leukemia.

Erythremia is treated with:

Bloodletting, in which ml of blood is removed to reduce blood viscosity in young people and 100 ml of blood in case of concomitant heart diseases or in the elderly. The course consists of 3 procedures, which are carried out at intervals of 2-3 days. Before the procedure, the patient takes medications that reduce blood clotting. Bloodletting is not performed in the presence of recent thrombosis.
Hardware treatment methods (erythrocytapheresis), which remove excess red blood cells and platelets. The procedure is carried out at intervals of 5-7 days.
Chemotherapy, which is used at stage II B, in the presence of an increase in the number of all blood cells, poor tolerance to bloodletting, or the presence of complications from internal organs or blood vessels. Chemotherapy is carried out according to a special regimen.
Symptomatic therapy, including antihypertensive drugs for high blood pressure (ACE inhibitors are usually prescribed), antihistamines to reduce skin itching, antiplatelet agents that reduce blood clotting, hemostatic drugs for bleeding.

To prevent thrombosis, anticoagulants are used (usually acetylsalicylic acid is prescribed pomg/day).

Nutrition for erythremia must meet the requirements of the treatment table according to Pevzner No. 6 (the amount of protein foods is reduced, red fruits and vegetables and foods containing dyes are excluded).

Content

Hematologists know that this disease is difficult to treat and has dangerous complications. Polycythemia is characterized by changes in the composition of the blood that affect the patient's health. How does the pathology develop, what symptoms is it characterized by? Find out diagnostic methods, treatment methods, medications, life prognosis for the patient.

What is polycythemia

Men are more susceptible to the disease than women; middle-aged people are more often affected. Polycythemia is an autosomal recessive pathology in which, for various reasons, the number of red blood cells in the blood increases. The disease has other names - erythrocytosis, multiblood, Vaquez disease, erythremia, its ICD-10 code is D45. The disease is characterized by:

splenomegaly – a significant increase in the size of the spleen;
increased blood viscosity;
significant production of leukocytes, platelets;
increase in circulating blood volume (CBV).

Polycythemia belongs to the group of chronic leukemias and is considered a rare form of leukemia. True erythremia (polycythemia vera) is divided into types:

Primary is a malignant disease with a progressive form associated with hyperplasia of the cellular components of the bone marrow - myeloproliferation. The pathology affects the erythroblastic germ, which causes an increase in the number of red blood cells.
Secondary polycythemia is a compensatory reaction to hypoxia caused by smoking, high altitude climbing, adrenal tumors, and pulmonary pathology.

Vaquez disease is dangerous due to complications. Due to high viscosity, blood circulation in peripheral vessels is impaired. Uric acid is released in large quantities. All this is fraught with:

bleeding;
thrombosis;
oxygen starvation of tissues;
hemorrhages;
hyperemia;
hemorrhage;
trophic ulcers;
renal colic;
ulcers in the gastrointestinal tract;
kidney stones;
splenomegaly;
gout;
myelofibrosis;
iron deficiency anemia;
myocardial infarction;
stroke;
fatal.

Types of disease

Vaquez disease, depending on development factors, is divided into types. Each has its own symptoms and treatment features. Doctors highlight:

polycythemia vera, which is caused by the appearance of a tumor substrate in the red bone marrow, leading to an increase in the production of red blood cells;
secondary erythremia - its cause is oxygen starvation, pathological processes occurring in the patient’s body and causing a compensatory reaction.

Primary

The disease is characterized by tumor origin. Primary polycythemia - myeloproliferative blood cancer - occurs when pluripotent stem cells of the bone marrow are damaged. When there is a disease in the patient’s body:

the activity of erythropoietin, which regulates the production of blood cells, increases;
the number of red blood cells, leukocytes, platelets increases;
synthesis of mutated brain cells occurs;
proliferation of infected tissues occurs;
a compensatory reaction to hypoxia occurs - there is an additional increase in the number of red blood cells.

With this type of pathology, it is difficult to influence mutated cells that have a high ability to divide. Thrombotic and hemorrhagic lesions appear. Vaquez disease has developmental features:

changes occur in the liver and spleen;
tissues are filled with viscous blood, prone to the formation of blood clots;
plethoric syndrome develops - cherry-red color of the skin;
severe itching occurs;
blood pressure (BP) increases;
hypoxia develops.

Polycythemia vera is dangerous due to its malignant development, causing severe complications. This form of pathology is characterized by the following stages:

Initial – lasts about five years, is asymptomatic, the size of the spleen is not changed. BCC increased slightly.
The advanced stage lasts up to 20 years. It is characterized by an increased content of red blood cells, platelets, and leukocytes. It has two substages - without changes in the spleen and with the presence of myeloid metaplasia.

The last stage of the disease – post-erythremic (anemic) – is characterized by complications:

secondary myelofibrosis;
leukopenia;
thrombocytopenia;
myeloid transformation of the liver, spleen;
cholelithiasis, urolithiasis;
transient ischemic attacks;
anemia - the result of bone marrow depletion;
pulmonary embolism;
myocardial infarction;
nephrosclerosis;
leukemia in acute, chronic form;
cerebral hemorrhages.

Secondary polycythemia (relative)

This form of Vaquez disease is provoked by external and internal factors. With the development of secondary polycythemia, viscous blood with increased volumes fills the vessels, provoking the formation of blood clots. With oxygen starvation of tissues, a compensation process develops:

the kidneys begin to intensively produce the hormone erythropoietin;
active synthesis of red blood cells in the bone marrow is triggered.

Secondary polycythemia occurs in two forms. Each of them has its own characteristics. The following varieties are distinguished:

stressful – caused by an unhealthy lifestyle, prolonged overexertion, nervous disorders, unfavorable working conditions;
false, in which the total number of red blood cells, leukocytes, platelets in the tests is within the normal range, an increase in ESR causes a decrease in plasma volume.

Causes

The provoking factors for the development of the disease depend on the form of the disease. Primary polycythemia occurs as a result of a neoplastic neoplasm of the red bone marrow. The predetermining causes of true erythrocytosis are:

genetic malfunctions in the body - mutation of the tyrosine kinase enzyme, when the amino acid valine is replaced by phenylalanine;
hereditary predisposition;
bone marrow cancers;
oxygen deficiency - hypoxia.

The secondary form of erythrocytosis is caused by external causes. Concomitant diseases play an equally important role in development. Provoking factors are:

climatic conditions;
living in high mountains;
congestive heart failure;
cancerous tumors of internal organs;
pulmonary hypertension;
action of toxic substances;
overstrain of the body;
x-ray radiation;
insufficient oxygen supply to the kidneys;
infections that cause intoxication of the body;
smoking;
bad ecology;
genetic features - Europeans are more likely to get sick.

The secondary form of Vaquez disease is caused by congenital causes - autonomous production of erythropoietin, high affinity of hemoglobin for oxygen. There are also acquired factors in the development of the disease:

arterial hypoxemia;
kidney pathologies - cystic lesions, tumors, hydronephrosis, renal artery stenosis;
bronchial carcinoma;
adrenal tumors;
cerebellar hemangioblastoma;
hepatitis;
cirrhosis of the liver;
tuberculosis.

Symptoms of Vaquez disease

The disease, caused by an increase in the number of red blood cells and blood volume, is distinguished by characteristic symptoms. They have their own characteristics depending on the stage of Vaquez's disease. General symptoms of pathology are observed:

dizziness;
visual impairment;
Cooperman's symptom - a bluish tint of the mucous membranes and skin;
angina attacks;
redness of the fingers of the lower and upper extremities, accompanied by pain and burning;
thrombosis of various localizations;
severe itching of the skin, aggravated by contact with water.

As the pathology progresses, the patient experiences pain syndromes of various localizations. Disorders of the nervous system are observed. The disease is characterized by:

weakness;
fatigue;
temperature increase;
enlarged spleen;
noise in ears;
dyspnea;
feeling of loss of consciousness;
plethoric syndrome - burgundy-red color of the skin;
headache;
vomit;
increased blood pressure;
pain in the hands from touching;
chilliness of the limbs;
redness of the eyes;
insomnia;
pain in the hypochondrium, bones;
pulmonary embolism.

initial stage

The disease is difficult to diagnose at the very beginning of its development. The symptoms are mild, similar to a cold or a condition of elderly people corresponding to old age. Pathology is detected accidentally during tests. Symptoms indicate the initial stage of erythrocytosis:

dizziness;
decreased visual acuity;
attacks of headache;
insomnia;
noise in ears;
soreness of fingers from touching;
cold extremities;
ischemic pain;
redness of mucous surfaces and skin.

Expanded (erythremic)

The development of the disease is characterized by the appearance of pronounced signs of high blood viscosity. Pancytosis is noted - an increase in the number of components in analyzes - red blood cells, leukocytes, platelets. The advanced stage is characterized by the presence of:

redness of the skin to purple shades;
telangiectasia - pinpoint hemorrhages;
acute attacks of pain;
itching, which intensifies when interacting with water.

At this stage of the disease, signs of iron deficiency are observed - split nails, dry skin. A characteristic symptom is a strong increase in the size of the liver and spleen. Patients experience:

indigestion;
breathing disorder;
arterial hypertension;
joint pain;
hemorrhagic syndrome;
microthrombosis;
ulcers of the stomach, duodenum;
bleeding;
cardialgia – pain in the left chest;
migraine.

In the advanced stage of erythrocytosis, patients complain of lack of appetite. Investigations reveal stones in the gall bladder. The disease is different:

increased bleeding from small cuts;
disturbances of rhythm and conduction of the heart;
swelling;
signs of gout;
pain in the heart;
microcytosis;
symptoms of urolithiasis;
changes in taste, smell;
bruises on the skin;
trophic ulcers;
renal colic.

Anemic stage

At this stage of development, the disease enters the terminal stage. The body does not have enough hemoglobin to function normally. The patient has:

significant enlargement of the liver;
progression of splenomegaly;
thickening of the spleen tissue;
with hardware examination – cicatricial changes in the bone marrow;
vascular thrombosis of deep veins, coronary, cerebral arteries.

At the anemic stage, the development of leukemia poses a danger to the patient’s life. This stage of Vaquez's disease is characterized by the occurrence of aplastic iron deficiency anemia, the cause of which is the displacement of hematopoietic cells from the bone marrow by connective tissue. In this case, symptoms are observed:

general weakness;
fainting;
feeling of lack of air.

At this stage, if left untreated, the patient will quickly die. Thrombotic and hemorrhagic complications lead to it:

ischemic form of stroke;
pulmonary embolism;
myocardial infarction;
spontaneous bleeding – gastrointestinal, esophageal veins;
cardiosclerosis;
arterial hypertension;
heart failure.

Symptoms of the disease in newborns

If the fetus has suffered hypoxia during the period of intrauterine development, its body in response begins to increase the production of red blood cells. The provoking factor for the appearance of erythrocytosis in infants is congenital heart disease and pulmonary pathologies. The disease leads to the following consequences:

formation of bone marrow sclerosis;
disruption of the production of leukocytes responsible for the newborn’s immune system;
development of infections leading to death.

At the initial stage, the disease is detected by test results - the level of hemoglobin, hematocrit, and red blood cells. As the pathology progresses, pronounced symptoms are observed already in the second week after birth:

the baby cries when touched;
the skin turns red;
the size of the liver and spleen increases;
thrombosis appears;
body weight decreases;
tests reveal an increased number of red blood cells, leukocytes, and platelets.

Diagnosis of polycythemia

Communication between a patient and a hematologist begins with a conversation, an external examination, and an anamnesis. The doctor finds out heredity, features of the course of the disease, the presence of pain, frequent bleeding, and signs of thrombosis. During admission, the patient is diagnosed with polycythaemic syndrome:

purplish-red blush;
intense coloring of the mucous membranes of the mouth and nose;
cyanotic (blue) color of the palate;
change in the shape of the fingers;
red eyes;
palpation reveals an increase in the size of the spleen and liver.

The next stage of diagnosis is laboratory tests. Indicators that indicate the development of the disease:

an increase in the total mass of red blood cells in the blood;
increased number of platelets, leukocytes;
significant levels of alkaline phosphatase;
a large amount of vitamin B 12 in the blood serum;
increased erythropoietin in secondary polycythemia;
decrease in situration (blood oxygen saturation) – less than 92%;
decrease in ESR;
increase in hemoglobin to 240 g/l.

For differential diagnosis of pathology, special types of studies and analyzes are used. Consultations with a urologist, cardiologist, and gastroenterologist are provided. The doctor prescribes:

biochemical blood test - determines the level of uric acid, alkaline phosphatase;
radiological examination - reveals an increase in circulating red blood cells;
sternal puncture - collection of bone marrow from the sternum for cytological analysis;
trephine biopsy - histology of tissue from the ilium, revealing three-line hyperplasia;
molecular genetic analysis.

Laboratory research

Polycythemia disease is confirmed by hematological changes in blood parameters. There are parameters that characterize the development of pathology. Laboratory data indicating the presence of polycythemia:

Index	Units	Meaning
Hemoglobin
Circulating red blood cell mass
Erythrocytosis	cells/liter
Leukocytosis		more than 12x109
Thrombocytosis		over 400x109
Hematocrit

Serum vitamin B 12 level
Alkaline phosphatase		more than 100
Color indicator

Hardware diagnostics

After laboratory tests, hematologists prescribe additional tests. To assess the risk of developing metabolic and thrombohemorrhagic disorders, hardware diagnostics are used. The patient undergoes studies depending on the characteristics of the disease. A patient with polycythemia is given:

Ultrasound of the spleen, kidneys;
heart examination - echocardiography.

Hardware diagnostic methods help assess the condition of blood vessels, identify the presence of bleeding and ulcers. Appointed:

fibrogastroduodenoscopy (FGDS) – instrumental study of the mucous membranes of the stomach and duodenum;
Doppler ultrasound (USDG) of the vessels of the neck, head, veins of the extremities;
computed tomography of internal organs.

Treatment of polycythemia

Before starting therapeutic measures, it is necessary to find out the type of disease and its causes - the treatment regimen depends on this. Hematologists are faced with the task of:

in case of primary polycythemia, prevent tumor activity by affecting the tumor in the bone marrow;
in the secondary form, identify the disease that provoked the pathology and eliminate it.

Treatment of polycythemia includes drawing up a rehabilitation and prevention plan for a specific patient. Therapy involves:

bloodletting, reducing the number of red blood cells to normal - 500 ml of blood is taken from the patient every two days;
maintaining physical activity;
erythocytophoresis – taking blood from a vein, followed by filtration and return to the patient;
diet;
transfusion of blood and its components;
chemotherapy to prevent leukemia.

In difficult situations that threaten the patient’s life, a bone marrow transplant is performed, splenectomy is the removal of the spleen. In the treatment of polycythemia, much attention is paid to the use of medications. The treatment regimen includes the use of:

corticosteroid hormones - in severe cases of the disease;
cytostatic agents - Hydroxyurea, Imiphos, which reduce the proliferation of malignant cells;
antiplatelet agents, blood thinners - Dipyridamole, Aspirin;
Interferon, which increases defenses and enhances the effectiveness of cytostatics.

Symptomatic treatment involves the use of drugs that reduce blood viscosity, prevent thrombosis, and the development of bleeding. Hematologists prescribe:

to exclude vascular thrombosis - Heparin;
for severe bleeding - Aminocaproic acid;
in case of erythromelalgia - pain in the fingertips - non-steroidal anti-inflammatory drugs - Voltaren, Indomethacin;
for itchy skin - antihistamines - Suprastin, Loratadine;
in case of infectious genesis of the disease - antibiotics;
for hypoxic reasons - oxygen therapy.

Phlebotomy or erythrocytopheresis

An effective treatment for polycythemia is phlebotomy. When performing bloodletting, the volume of circulating blood decreases, the number of red blood cells (hematocrit) decreases, and skin itching is eliminated. Features of the process:

before phlebotomy, the patient is administered Heparin or Reopoliglucin to improve microcirculation and blood fluidity;
excess is removed using leeches or an incision is made to puncture the vein;
up to 500 ml of blood is removed at a time;
the procedure is carried out at intervals of 2 to 4 days;
hemoglobin is reduced to 150 g/l;
hematocrit is adjusted to 45%.

Another method of treating polycythemia, erythrocytopheresis, is more effective. During extracorporeal hemocorrection, excess red blood cells are removed from the patient's blood. This improves hematopoietic processes and increases iron consumption by the bone marrow. Scheme for performing cytopheresis:

They create a vicious circle - the patient’s veins of both arms are connected through a special device.
Blood is taken from one.P
It is passed through a machine with a centrifuge, separator, and filters, where some of the red blood cells are removed.
The purified plasma is returned to the patient and injected into a vein in the other arm.

Myelosuppressive therapy with cytostatics

In severe cases of polycythemia, when bloodletting does not produce positive results, doctors prescribe drugs that suppress the formation and reproduction of brain cells. Treatment with cytostatics requires constant blood tests to monitor the effectiveness of therapy. Indications are factors accompanying polycythaemic syndrome:

visceral, vascular complications;
skin itching;
splenomegaly;
thrombocytosis;
leukocytosis.

Hematologists prescribe medications taking into account test results and the clinical picture of the disease. Contraindications for cytostatic therapy are childhood. The following medications are used to treat polycythemia:

Myelobramol;
Imifos;
Cyclophosphamide;
Alkeran;
Myelosan;
Hydroxyurea;
Cyclophosphamide;
Mitobronitol;
Busulfan.

Preparations for normalizing the state of blood aggregation

The objectives of treatment for polycythemia: normalization of hematopoiesis, which includes ensuring the liquid state of the blood, its coagulation during bleeding, and restoration of the walls of blood vessels. Doctors face a serious choice of drugs so as not to harm the patient. Prescribed medications that help stop bleeding - hemostatics:

coagulants – Thrombin, Vikasol;
fibrinolysis inhibitors – Kontrikal, Ambien;
stimulators of vascular aggregation – Calcium chloride;
drugs that reduce permeability - Rutin, Adroxon.

The use of antithrombotic agents is of great importance in the treatment of polycythemia to restore the aggregative state of the blood:

anticoagulants - Heparin, Girudin, Phenilin;
fibronolytics – Streptolyasis, Fibrinolysin;
antiplatelet agents: platelet - Aspirin (Acetylsalicylic acid), Dipyridamole, Indobrufen; erythrocytes - Reogluman, Reopoliglucin, Pentoxifylline.

Recovery prognosis

What awaits a patient diagnosed with polycythemia? Forecasts depend on the type of disease, timely diagnosis and treatment, causes, and the occurrence of complications. Vaquez disease in its primary form has an unfavorable development scenario. Life expectancy is up to two years, which is associated with the complexity of therapy, high risks of strokes, heart attacks, and thromboembolic consequences. Survival can be increased by using the following treatments:

local irradiation of the spleen with radioactive phosphorus;
lifelong phlebotomy procedures;
chemotherapy.

A more favorable prognosis for the secondary form of polycythemia, although the disease can result in nephrosclerosis, myelofibrosis, and erythrocyanosis. Although a complete cure is impossible, the patient’s life is extended for a significant period - over fifteen years - provided:

constant monitoring by a hematologist;
cytostatic treatment;
regular hemocorrection;
undergoing chemotherapy;
eliminating factors that provoke the development of the disease;
treatment of pathologies that caused the disease.

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ICD-10 was introduced into healthcare practice throughout the Russian Federation in 1999 by order of the Russian Ministry of Health dated May 27, 1997. No. 170

The release of a new revision (ICD-11) is planned by WHO in 2017-2018.

With changes and additions from WHO.

Processing and translation of changes © mkb-10.com

Secondary polycythemia

Definition and general information [edit]

Synonyms: secondary erythrocytosis

Etiology and pathogenesis[edit]

Clinical manifestations[edit]

Clinical features vary depending on the etiology of polycythemia, but typically symptoms may include plethora, flushed complexion, headache, and tinnitus. The congenital form may be accompanied by thrombophlebitis of the superficial or deep veins, may be associated with specific symptoms, as in the case of Chuvash familial erythrocytosis, or the course of the disease may be indolent.

The acquired form of secondary polycythemia can manifest as cyanosis, hypertension, drumsticks on the legs and arms, and drowsiness.

Secondary polycythemia: Diagnosis [edit]

Differential diagnosis[edit]

Secondary polycythemia: Treatment[edit]

The prognosis mainly depends on the concomitant disease in acquired forms of secondary erythrocytosis and the severity of thrombotic complications in hereditary forms, such as Chuvash erythrocytosis.

Prevention[edit]

Other [edit]

Synonyms: stress erythrocytosis, stress polycythemia, stress polycythemia

Heisbock syndrome is characterized by secondary polycythemia and occurs mainly in men on a high-calorie diet.

The prevalence of Heisbock syndrome is unknown.

The prognosis is worsened by the development of cardiovascular complications.

Polycythemia

ICD-10 code

Titles

Description

Symptoms

There are several stages in the clinical course:

*initial, or asymptomatic, stage, usually lasting 5 years, with minimal clinical manifestations;

*stage IIA - erythraemic advanced stage, without myeloid metaplasia of the spleen, its duration can reach years;

*stage IIB - erythremic advanced stage, with myeloid metaplasia of the spleen;.

*stage III - stage of post-erythraemic myeloid metaplasia (anemic stage) with or without myelofibrosis; possible outcome in acute leukemia, chronic myeloid leukemia.

However, given the usual onset of the disease in elderly and old people, not all patients go through all three stages.

Hemorrhagic syndrome is manifested by spontaneous bleeding of the gums, nosebleeds, ecchymoses, characteristic of disorders of the platelet-vascular component of hemostasis.

Pathogenesis

Causes

The main causes of secondary erythrocytosis include tissue hypoxia, both congenital and acquired, and changes in the content of endogenous erythropoietin.

Causes of secondary erythrocytosis:

1,high affinity of hemoglobin for oxygen;.

2,low level of 2,3-diphosphoglycerate;.

3,autonomous production of erythropoietin.

1, arterial hypoxemia of physiological and pathological nature:

“blue” heart defects;.

Chronic pulmonary diseases;

Adaptation to high mountain conditions.

Diffuse diseases of the renal parenchyma;

Renal artery stenosis.

Treatment

Planned therapy. Modern therapy for erythremia consists of the use of blood exfusions, cytostatic drugs, the use of radioactive phosphorus, and interferon.

The following drugs are used to treat erythremia:

*alkylating agents - myelosan, alkeran, cyclophosphamide.

To improve the quality of life, patients are given symptomatic therapy:

*uric acid diathesis (with clinical manifestations of urolithiasis, gout) requires constant intake of allopurinol (miluritis) in a daily dose of 200 mg to 1 g;

*erythromelalgia is an indication for prescribing 500 mg of aspirin or 250 mg of methindol; for severe erythromelalgia, additional heparin is indicated;

*skin itching is somewhat relieved by antihistamines; interferon has a significant, but slower (not earlier than 2 months) effect.

ICD 10. Class III (D50-D89)

ICD 10. Class III. Diseases of the blood, hematopoietic organs and certain disorders involving the immune mechanism (D50-D89)

Excluded: autoimmune disease (systemic) NOS (M35.9), certain conditions arising in the perinatal period (P00-P96), complications of pregnancy, childbirth and the puerperium (O00-O99), congenital anomalies, deformities and chromosomal disorders (Q00- Q99), endocrine diseases, nutritional and metabolic disorders (E00-E90), disease caused by human immunodeficiency virus [HIV] (B20-B24), trauma, poisoning and certain other consequences of external causes (S00-T98), neoplasms (C00-D48), symptoms, signs and abnormalities identified by clinical and laboratory tests, not classified elsewhere (R00-R99)

This class contains the following blocks:

D50-D53 Anemia associated with nutrition

D55-D59 Hemolytic anemias

D60-D64 Aplastic and other anemias

D65-D69 Bleeding disorders, purpura and other hemorrhagic conditions

D70-D77 Other diseases of the blood and hematopoietic organs

D80-D89 Selected disorders involving the immune mechanism

The following categories are marked with an asterisk:

D77 Other disorders of the blood and hematopoietic organs in diseases classified elsewhere

NUTRITION-RELATED ANEMIA (D50-D53)

D50 Iron deficiency anemia

D50.0 Iron deficiency anemia secondary to blood loss (chronic). Posthemorrhagic (chronic) anemia.

Excludes: acute posthemorrhagic anemia (D62) congenital anemia due to fetal blood loss (P61.3)

D50.1 Sideropenic dysphagia. Kelly-Paterson syndrome. Plummer-Vinson syndrome

D51 Vitamin B12 deficiency anemia

Excludes: vitamin B12 deficiency (E53.8)

D51.0 Vitamin B12 deficiency anemia due to intrinsic factor deficiency.

Congenital intrinsic factor deficiency

D51.1 Vitamin B12 deficiency anemia due to selective malabsorption of vitamin B12 with proteinuria.

Imerslund(-Gresbeck) syndrome. Megaloblastic hereditary anemia

D51.3 Other vitamin B12 deficiency anemias associated with nutrition. Anemia of vegetarians

D51.8 Other vitamin B12 deficiency anemias

D51.9 Vitamin B12 deficiency anemia, unspecified

D52 Folate deficiency anemia

D52.0 Folate deficiency anemia associated with nutrition. Megaloblastic nutritional anemia

D52.1 Folate deficiency anemia, drug-induced. If necessary, identify the drug

use an additional external cause code (class XX)

D52.9 Folate deficiency anemia, unspecified. Anemia due to insufficient intake of folic acid, NOS

D53 Other diet-related anemias

Includes: megaloblastic anemia not responding to vitamin therapy

nom B12 or folate

D53.0 Anemia due to protein deficiency. Anemia due to amino acid deficiency.

Excludes: Lesch-Nychen syndrome (E79.1)

D53.1 Other megaloblastic anemias, not elsewhere classified. Megaloblastic anemia NOS.

Excludes: DiGuglielmo disease (C94.0)

Excludes: scurvy (E54)

D53.8 Other specified anemias associated with nutrition.

Anemia associated with deficiency:

Excludes: malnutrition without mention of

anemia, such as:

Copper deficiency (E61.0)

Molybdenum deficiency (E61.5)

Zinc deficiency (E60)

D53.9 Diet-related anemia, unspecified. Simple chronic anemia.

Excludes: anemia NOS (D64.9)

HEMOLYTIC ANEMIA (D55-D59)

D55 Anemia due to enzyme disorders

Excludes: drug-induced enzyme deficiency anemia (D59.2)

D55.0 Anemia due to glucose-6-phosphate dehydrogenase [G-6-PD] deficiency. Favism. G-6-PD deficiency anemia

D55.1 Anemia due to other disorders of glutathione metabolism.

Anemia due to deficiency of enzymes (except G-6-PD) associated with hexose monophosphate [HMP]

bypass of the metabolic pathway. Hemolytic nonspherocytic anemia (hereditary) type 1

D55.2 Anemia due to disorders of glycolytic enzymes.

Hemolytic non-spherocytic (hereditary) type II

Due to hexokinase deficiency

Due to pyruvate kinase deficiency

Due to triosephosphate isomerase deficiency

D56 Thalassemia

Excludes: hydrops fetalis due to hemolytic disease (P56.-)

D56.1 Beta thalassemia. Cooley's anemia. Severe beta thalassemia. Sickle cell beta thalassemia.

D56.9 Thalassemia, unspecified. Mediterranean anemia (with other hemoglobinopathy)

Thalassemia minor (mixed) (with other hemoglobinopathy)

D57 Sickle cell disorders

Excludes: other hemoglobinopathies (D58. -)

sickle cell beta thalassemia (D56.1)

D57.0 Sickle cell anemia with crisis. Hb-SS disease with crisis

D57.1 Sickle cell anemia without crisis.

D57.2 Double heterozygous sickle cell disorders

D57.3 Carriage of the sickle cell trait. Carriage of hemoglobin S. Heterozygous hemoglobin S

D58 Other hereditary hemolytic anemias

D58.0 Hereditary spherocytosis. Acholuric (familial) jaundice.

Congenital (spherocytic) hemolytic jaundice. Minkowski-Choffard syndrome

D58.1 Hereditary elliptocytosis. Ellitocytosis (congenital). Ovalocytosis (congenital) (hereditary)

D58.2 Other hemoglobinopathies. Abnormal hemoglobin NOS. Congenital anemia with Heinz bodies.

Hemolytic disease caused by unstable hemoglobin. Hemoglobinopathy NOS.

Excludes: familial polycythemia (D75.0)

Hb-M disease (D74.0)

hereditary persistence of fetal hemoglobin (D56.4)

altitude-related polycythemia (D75.1)

D58.9 Hereditary hemolytic anemia, unspecified

D59 Acquired hemolytic anemia

D59.0 Drug-induced autoimmune hemolytic anemia.

If it is necessary to identify the drug, use an additional code for external causes (class XX).

D59.1 Other autoimmune hemolytic anemias. Autoimmune hemolytic disease (cold type) (warm type). Chronic disease caused by cold hemagglutinins.

Cold type (secondary) (symptomatic)

Thermal type (secondary) (symptomatic)

Excludes: Evans syndrome (D69.3)

hemolytic disease of the fetus and newborn (P55. -)

paroxysmal cold hemoglobinuria (D59.6)

D59.2 Drug-induced non-autoimmune hemolytic anemia. Drug-induced enzyme deficiency anemia.

If it is necessary to identify the drug, use an additional code for external causes (class XX).

D59.4 Other non-autoimmune hemolytic anemias.

If it is necessary to identify the cause, use an additional external cause code (class XX).

D59.5 Paroxysmal nocturnal hemoglobinuria [Marchiafava-Micheli].

D59.6 Hemoglobinuria due to hemolysis caused by other external causes.

Excludes: hemoglobinuria NOS (R82.3)

D59.9 Acquired hemolytic anemia, unspecified. Chronic idiopathic hemolytic anemia

APLASTIC AND OTHER ANEMIA (D60-D64)

D60 Acquired pure red cell aplasia (erythroblastopenia)

Includes: red cell aplasia (acquired) (adults) (with thymoma)

D60.9 Acquired pure red cell aplasia, unspecified

D61 Other aplastic anemias

Excluded: agranulocytosis (D70)

D61.0 Constitutional aplastic anemia.

Aplasia (pure) red cell:

Blackfan-Diamond syndrome. Familial hypoplastic anemia. Fanconi anemia. Pancytopenia with developmental defects

D61.1 Drug-induced aplastic anemia. If necessary, identify the drug

use an additional code for external causes (class XX).

D61.2 Aplastic anemia caused by other external agents.

If it is necessary to identify the cause, use an additional code of external causes (class XX).

D61.9 Aplastic anemia, unspecified. Hypoplastic anemia NOS. Bone marrow hypoplasia. Panmyelophthisis

D62 Acute posthemorrhagic anemia

Excludes: congenital anemia due to fetal blood loss (P61.3)

D63 Anemia in chronic diseases classified elsewhere

D63.0 Anemia due to neoplasms (C00-D48+)

D63.8 Anemia in other chronic diseases classified elsewhere

D64 Other anemias

Excluded: refractory anemia:

With excess blasts (D46.2)

With transformation (D46.3)

With sideroblasts (D46.1)

No sideroblasts (D46.0)

D64.1 Secondary sideroblastic anemia due to other diseases.

If necessary, an additional code is used to identify the disease.

D64.2 Secondary sideroblastic anemia caused by drugs or toxins.

If it is necessary to identify the cause, use an additional code of external causes (class XX).

D64.3 Other sideroblastic anemias.

Pyridoxine-reactive, not elsewhere classified

Excludes: Blackfan-Diamond syndrome (D61.0)

DiGuglielmo disease (C94.0)

BLOOD CLOTTING DISORDERS, PURPURA AND OTHERS

HEMORRHAGIC CONDITIONS (D65-D69)

D65 Disseminated intravascular coagulation [defibration syndrome]

Afibrinogenemia acquired. Consumptive coagulopathy

Diffuse or disseminated intravascular coagulation

Acquired fibrinolytic bleeding

Excluded: defibration syndrome (complicating):

In a newborn (P60)

D66 Hereditary factor VIII deficiency

Factor VIII deficiency (with functional impairment)

D67 Hereditary factor IX deficiency

Factor IX (with functional impairment)

Thromboplastic plasma component

D68 Other bleeding disorders

Abortion, ectopic or molar pregnancy (O00-O07, O08.1)

Pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)

Excludes: hereditary capillary fragility (D69.8)

factor VIII deficiency:

With functional impairment (D66)

D68.1 Hereditary factor XI deficiency. Hemophilia C. Plasma thromboplastin precursor deficiency

D68.2 Hereditary deficiency of other coagulation factors. Congenital afibrinogenemia.

Dysfibrinogenemia (congenital). Hypoproconvertinemia. Ovren's disease

D68.3 Hemorrhagic disorders caused by anticoagulants circulating in the blood. Hyperheparinemia.

If necessary, identify the anticoagulant used, use an additional external cause code.

D68.4 Acquired coagulation factor deficiency.

Coagulation factor deficiency due to:

Vitamin K deficiency

Excludes: vitamin K deficiency in the newborn (P53)

D69 Purpura and other hemorrhagic conditions

Excludes: benign hypergammaglobulinemic purpura (D89.0)

cryoglobulinemic purpura (D89.1)

idiopathic (hemorrhagic) thrombocythemia (D47.3)

lightning purple (D65)

thrombotic thrombocytopenic purpura (M31.1)

D69.0 Allergic purpura.

D69.1 Qualitative platelet defects. Bernard-Soulier syndrome [giant platelets].

Glanzmann's disease. Gray platelet syndrome. Thrombasthenia (hemorrhagic) (hereditary). Thrombocytopathy.

D69.2 Other non-thrombocytopenic purpura.

Excludes: thrombocytopenia with absent radius (Q87.2)

transient neonatal thrombocytopenia (P61.0)

Wiskott-Aldrich syndrome (D82.0)

D69.5 Secondary thrombocytopenia. If it is necessary to identify the cause, use an additional external cause code (class XX).

D69.8 Other specified hemorrhagic conditions. Capillary fragility (hereditary). Vascular pseudohemophilia

OTHER DISEASES OF THE BLOOD AND BLOOD FORMING ORGANS (D70-D77)

D70 Agranulocytosis

Agranulocytic tonsillitis. Children's genetic agranulocytosis. Kostmann's disease

If it is necessary to identify the drug causing the neutropenia, use an additional external cause code (class XX).

D71 Functional disorders of polymorphonuclear neutrophils

Defect of the cell membrane receptor complex. Chronic (children's) granulomatosis. Congenital dysphagocytosis

Progressive septic granulomatosis

D72 Other white blood cell disorders

Excludes: basophilia (D75.8)

immune disorders (D80-D89)

preleukemia (syndrome) (D46.9)

D72.0 Genetic abnormalities of leukocytes.

Anomaly (granulation) (granulocyte) or syndrome:

Excluded: Chediak-Higashi (-Steinbrink) syndrome (E70.3)

D72.8 Other specified white blood cell disorders.

Leukocytosis. Lymphocytosis (symptomatic). Lymphopenia. Monocytosis (symptomatic). Plasmacytosis

D72.9 White blood cell disorder, unspecified

D73 Diseases of the spleen

D73.8 Other diseases of the spleen. Splenic fibrosis NOS. Perisplenitis. Splenitis NOS

D74 Methemoglobinemia

D74.0 Congenital methemoglobinemia. Congenital deficiency of NADH-methemoglobin reductase.

Hemoglobinosis M [Hb-M disease]. Hereditary methemoglobinemia

D74.8 Other methemoglobinemia. Acquired methemoglobinemia (with sulfhemoglobinemia).

Toxic methemoglobinemia. If it is necessary to identify the cause, use an additional external cause code (class XX).

D74.9 Methemoglobinemia, unspecified

Excludes: swollen lymph nodes (R59. -)

hypergammaglobulinemia NOS (D89.2)

Mesenteric (acute) (chronic) (I88.0)

D75.1 Secondary polycythemia.

Decreased plasma volume

D75.2 Essential thrombocytosis.

Excludes: essential (hemorrhagic) thrombocythemia (D47.3)

D76 Selected diseases involving lymphoreticular tissue and the reticulohistiocytic system

Excludes: Letterer-Sieve disease (C96.0)

malignant histiocytosis (C96.1)

reticuloendotheliosis or reticulosis:

Histiocytic medullary (C96.1)

D76.0 Langerhans cell histiocytosis, not elsewhere classified. Eosinophilic granuloma.

Hand-Schueller-Crisgen disease. Histiocytosis X (chronic)

D76.1 Hemophagocytic lymphohistiocytosis. Familial hemophagocytic reticulosis.

Histiocytoses from mononuclear phagocytes other than Langerhans cells, NOS

D76.2 Hemophagocytic syndrome associated with infection.

If it is necessary to identify an infectious pathogen or disease, an additional code is used.

D76.3 Other histiocytosis syndromes. Reticulohistiocytoma (giant cell).

Sinus histiocytosis with massive lymphadenopathy. Xanthogranuloma

D77 Other disorders of the blood and hematopoietic organs in diseases classified elsewhere.

Splenic fibrosis in schistosomiasis [bilharzia] (B65. -)

SELECTED DISORDERS INVOLVING THE IMMUNE MECHANISM (D80-D89)

Includes: defects in the complement system, immunodeficiency disorders, excluding disease,

caused by human immunodeficiency virus [HIV] sarcoidosis

Excludes: autoimmune diseases (systemic) NOS (M35.9)

functional disorders of polymorphonuclear neutrophils (D71)

human immunodeficiency virus [HIV] disease (B20-B24)

D80 Immunodeficiencies with predominant antibody deficiency

D80.0 Hereditary hypogammaglobulinemia.

Autosomal recessive agammaglobulinemia (Swiss type).

X-linked agammaglobulinemia [Bruton] (with growth hormone deficiency)

D80.1 Non-familial hypogammaglobulinemia. Agammaglobulinemia with the presence of B-lymphocytes carrying immunoglobulins. General agammaglobulinemia. Hypogammaglobulinemia NOS

D80.2 Selective immunoglobulin A deficiency

D80.3 Selective deficiency of immunoglobulin G subclasses

D80.4 Selective immunoglobulin M deficiency

D80.5 Immunodeficiency with increased levels of immunoglobulin M

D80.6 Antibody deficiency with immunoglobulin levels close to normal or with hyperimmunoglobulinemia.

Antibody deficiency with hyperimmunoglobulinemia

D80.8 Other immunodeficiencies with a predominant antibody defect. Kappa light chain deficiency

D81 Combined immunodeficiencies

D81.0 Severe combined immunodeficiency with reticular dysgenesis

D81.1 Severe combined immunodeficiency with low T- and B-cell counts

D81.2 Severe combined immunodeficiency with low or normal B-cell count

D81.3 Adenosine deaminase deficiency

D81.5 Purine nucleoside phosphorylase deficiency

D81.6 Deficiency of class I molecules of the major histocompatibility complex. Naked lymphocyte syndrome

D81.7 Deficiency of class II molecules of the major histocompatibility complex

D81.8 Other combined immunodeficiencies. Biotin-dependent carboxylase deficiency

D81.9 Combined immunodeficiency, unspecified. Severe combined immunodeficiency disorder NOS

D82 Immunodeficiencies associated with other significant defects

Excludes: ataxic telangiectasia [Louis-Bart] (G11.3)

D82.0 Wiskott-Aldrich syndrome. Immunodeficiency with thrombocytopenia and eczema

D82.1 Di Georg syndrome. Pharyngeal diverticulum syndrome.

Aplasia or hypoplasia with immune deficiency

D82.3 Immunodeficiency due to a hereditary defect caused by the Epstein-Barr virus.

X-linked lymphoproliferative disease

D82.4 Hyperimmunoglobulin E syndrome

D83 Common variable immunodeficiency

D83.0 General variable immunodeficiency with predominant abnormalities in the number and functional activity of B cells

D83.1 General variable immunodeficiency with a predominance of disorders of immunoregulatory T cells

D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells

D83.8 Other common variable immunodeficiencies

D84 Other immunodeficiencies

D84.0 Lymphocyte functional antigen-1 defect

D84.1 Defect in the complement system. C1 esterase inhibitor deficiency

D84.8 Other specified immunodeficiency disorders

D86 Sarcoidosis

D86.8 Sarcoidosis of other specified and combined localizations. Iridocyclitis in sarcoidosis (H22.1).

Multiple cranial nerve palsies in sarcoidosis (G53.2)

Uveoparotitic fever [Herfordt's disease]

D86.9 Sarcoidosis, unspecified

D89 Other disorders involving the immune mechanism, not elsewhere classified

Excludes: hyperglobulinemia NOS (R77.1)

monoclonal gammopathy (D47.2)

non-engraftment and graft rejection (T86. -)

D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified

D75 Other diseases of the blood and hematopoietic organs

Excluded: enlarged lymph nodes (R59.-) hypergammaglobulinemia NOS (D89.2) lymphadenitis: . NOS (I88.9) . acute (L04.-) . chronic (I88.1). mesenteric (acute) (chronic) (I88.0)

D75.0 Familial erythrocytosis

Polycythemia: . benign. familial Excludes: hereditary ovalocytosis (D58.1)

D75.1 Secondary polycythemia

Polycythemia: . acquired. related to: . erythropoietins. decrease in plasma volume. height. stress. emotional. hypoxemic. nephrogenic. relative Excluded: polycythemia: . newborn (P61.1) . true (D45)

D75.2 Essential thrombocytosis

Excludes: essential (hemorrhagic) thrombocythemia (D47.3)

ICD-10: Class III. Diseases of the blood, hematopoietic organs and certain disorders involving the immune mechanism

Class III.

D50-D53

D55-D59

D60-D64

D65-D69

D70-D77

D80-D89

Diseases of the blood, hematopoietic organs and certain disorders involving the immune mechanism (D50 - D89)

Excludes: autoimmune disease (systemic) NOS (M35.9)

disease caused by the human immunodeficiency virus HIV (B20. - B24.) congenital anomalies (malformations), deformations and chromosomal disorders (Q00. - Q99.) neoplasms (C00. - D48.) complications of pregnancy, childbirth and the postpartum period (O00. - O99.) certain conditions arising in the perinatal period (P00. - P96.) symptoms, signs and deviations from the norm identified during clinical and laboratory tests, not classified elsewhere (R00. - R99.) injuries, poisoning and some other consequences of external causes (S00. - T98.) endocrine diseases, nutritional disorders and metabolic disorders (E00. - E90.).

Diet-related anemias (D50-D53)

D50.Iron deficiency anemia
- Anemias included: sideropenic and hypochromic
- D50.0 Iron deficiency anemia secondary to blood loss (chronic). Posthemorrhagic (chronic) anemia
- D50.1 Sideropenic dysphagia Kelly-Paterson syndrome, Plummer-Vinson syndrome
- D50.8 Other iron deficiency anemias
- D50.9 Iron deficiency anemia, unspecified
D51. Vitamin B12 deficiency anemia.
- Excludes: vitamin B12 deficiency (E53.8)
- D51.0 Vitamin B12 deficiency anemia due to intrinsic factor deficiency. Addison-Beermer Anemia, Pernicious Anemia (congenital), Congenital intrinsic factor deficiency
- D51.1 Vitamin B12 deficiency anemia due to selective malabsorption of vitamin B12 with proteinuria. Imerslund (- Gresbeck) syndrome, Megaloblastic hereditary anemia
- D51.2 Transcobalamin II deficiency
- D51.3 Other vitamin B12 deficiency anemias associated with nutrition. Anemia of vegetarians
- D51.8 Other vitamin BI2 deficiency anemias
- D51.9 Vitamin B12 deficiency anemia, unspecified
D52. Folate deficiency anemia
- D52.0 Folate deficiency anemia associated with nutrition. Megaloblastic nutritional anemia.
- D52.1 Folate deficiency anemia, drug-induced
- D52.8 Other folate deficiency anemias
D52.9 Folate deficiency anemia, unspecified. Anemia caused by insufficient intake of folic acid, NOS.
D53. Other diet-related anemias.
- Includes: megaloblastic anemia not responding to vitamin B12 or folate supplementation
- D53.0 Anemia due to protein deficiency. Anemia due to amino acid deficiency. Orotaciduric anemia.
- Excludes: Lesch-Nychen syndrome (E79.1)
- D53.1 Other megaloblastic anemias, not elsewhere classified. Megaloblastic anemia NOS. Excludes: DiGuglielmo disease (C94.0)
- D53.2 Anemia due to scurvy.
- Excludes: scurvy (E54)
- D53.8 Other specified anemias associated with nutrition. Anemia associated with deficiency: copper, molybdenum, zinc.

Excludes: malnutrition without mention of anemia, such as: copper deficiency (E61.0), molybdenum deficiency (E61.5) zinc deficiency (E60)

D53.9 Diet-related anemia, unspecified.

Hemolytic anemias (D55 - D59)

D55. Anemia due to enzyme disorders.
- Excludes: enzyme deficiency anemia caused by drugs (059.2)
- D55.0 Anemia due to glucose-6-phosphate dehydrogenase [G-6-PD] deficiency, Favism, G-6-PD deficiency anemia
- D55.1 Anemia due to other disorders of glutathione metabolism. Anemia due to enzyme deficiency (except for G-6-PD) associated with the hexose monophosphate [HMP] metabolic pathway shunt. Hemolytic nonspherocytic anemia (hereditary) type I.
- D55.2 Anemia due to disorders of glycolytic enzymes. Anemia: hemolytic non-spherocytic (hereditary) type II, due to hexokinase deficiency, due to pyruvate kinase deficiency, due to triosephosphate isomerase deficiency
- D55.3 Anemia due to disorders of nucleotide metabolism
- D55.8 Other anemia due to enzyme disorders
- D55.9 Anemia due to enzyme disorder, unspecified
D56. Thalassemia
- D56.0 Alpha thalassemia.
- Excludes: hydrops fetalis due to hemolytic disease (P56.-)
- D56.1 Beta thalassemia Cooley's anemia. Severe beta thalassemia. Sickle cell beta thalassemia. Thalassemia: intermediate, major
- D56.2 Delta-beta thalassemia
- D56.3 Carriage of thalassemia trait
- D56.4 Hereditary persistence of fetal hemoglobin [HFH]
- D56.8 Other thalassemias
- D56.9 Thalassemia, unspecified. Mediterranean anemia (with other hemoglobinopathy). Thalassemia minor (mixed) (with other hemoglobinopathy)
D57. Sickle cell disorders.
- Excluded: other hemoglobinopathies (D58.-) sickle cell beta thalassemia (D56.1)
- D57.0 Sickle cell anemia with crisis, Hb-SS disease with crisis
- D57.1 Sickle cell anemia without crisis. Sickle cell: anemia, disease, disorder.
- D57.2 Double heterozygous sickle cell disorders. Disease. Hb-SC. Hb-SD. Hb-SE.
- D57.3 Carriage of sickle cell trait. Carriage of hemoglobin S. Heterozygous hemoglobin S
- D57.8 Other sickle cell disorders
D58. Other hereditary hemolytic anemias
- D58.0 Hereditary spherocytosis. Acholuric (familial) jaundice. Congenital (spherocytic) hemolytic jaundice. Minkowski-Choffard syndrome
- D58.1 Hereditary elliptocytosis. Elliptocytosis (congenital). Ovalocytosis (congenital) (hereditary)
- D58.2 Other hemoglobinopathies. Abnormal hemoglobin NOS. Congenital anemia with Heinz bodies - Disease: H-C, H-D, H-E. Hemoglobinopathy NOS. Hemolytic disease caused by unstable hemoglobin.
- Excluded: familial polycythemia (D75.0), Hb-M disease (D74.0), hereditary persistence of fetal hemoglobin (D56.4), altitude-related polycythemia (D75.1), methemoglobinemia (D74.-)
- D58.8 Other specified hereditary hemolytic anemias. Stomatocytosis
- D58.9 Hereditary hemolytic anemia, unspecified
D59. Acquired hemolytic anemia
- D59.0 Drug-induced autoimmune hemolytic anemia
- D59.1 Other autoimmune hemolytic anemias. Autoimmune hemolytic disease (cold type) (warm type). Chronic disease caused by cold hemagglutinins. “Cold agglutinin”: disease, hemoglobinuria. Hemoltic anemia: cold type (secondary) (symptomatic), warm type (secondary) (symptomatic). Excluded: Evans syndrome (D69.3), hemolytic disease of the fetus and newborn (P55.-), paroxysmal cold hemoglobinuria (D59.6)
- D59.2 Drug-induced non-autoimmune hemolytic anemia. Drug-induced enzyme deficiency anemia
- D59.3 Hemolytic-uremic syndrome
- D59.4 Other non-autoimmune hemolytic anemias. Hemolytic anemia: mechanical, microangiopathic, toxic
- D59.5 Paroxysmal nocturnal hemoglobinuria (Marchiafava - Micheli).
- Excludes: hemoglobinuria NOS (R82.3)
- D59.6 Hemoglobinuria due to hemolysis caused by other external causes. Hemoglobinuria: from exertion, marching, paroxysmal cold.
- Excludes: hemoglobinuria NOS (R82.3)
D59.8 Other acquired hemolytic anemias
D59.9 Acquired hemolytic anemia, unspecified. Chronic idiopathic hemolytic anemia.

Aplastic and other anemias (D60-D64)

D60. Acquired pure red cell aplasia (erythroblastopenia).
- Includes: red cell aplasia (acquired) (adults) (with thymoma)
- D60.0 Chronic acquired pure red cell aplasia
- D60.1 Transient acquired pure red cell aplasia
- D60.8 Other acquired pure red cell aplasias
- D60.9 Acquired pure red cell aplasia, unspecified
D61. Other aplastic anemias.
- Excluded: agranulocytosis (D70.)
- D61.0 Constitutional aplastic anemia. Aplasia (pure) red cell: congenital, childhood, primary. Blackfan-Diamond syndrome. Familial hypoplastic anemia. Fanconi anemia. Pancytopenia with developmental defects
- D61.1 Drug-induced aplastic anemia. If it is necessary to identify the drug, use an additional code for external causes (class XX).
- D61.2 Aplastic anemia caused by other external agents. If it is necessary to identify the drug, use an additional code for external causes (class XX).
- D61.3 Idiopathic aplastic anemia
- D61.8 Other specified aplastic anemias
- D61.9 Aplastic anemia, unspecified. Hypoplastic anemia NOS. Bone marrow hypoplasia. Panmyelophthisis.
D62. Acute posthemorrhagic anemia.
- Excludes: congenital anemia due to fetal blood loss (P61.3)
D63. Anemia in chronic diseases classified elsewhere
- D63.0 Anemia due to neoplasms (C00 - D48)
- D63.8 Anemia in other chronic diseases classified elsewhere.
D64. Other anemias.
- Excluded: refractory anemia: NOS (D46.4), with excess blasts (D46.2), with transformation (D46.3), with sideroblasts (D46.1), without sideroblasts (D46.0).
- D64.0 Hereditary sideroblastic anemia. Sex-linked hypochromic sideroblastic anemia
- D64.1 Secondary sideroblastic anemia due to other diseases
- D64.2 Secondary sideroblastic anemia caused by drugs or toxins
- D64.3 Other sideroblastic anemias. Sideroblastic anemia: NOS, pyridoxine-responsive, not elsewhere classified
- D64.4 Congenital dyserythropoietic anemia. Dyshematopoietic anemia (congenital).
- Excluded: Blackfan-Diamond syndrome (D61.0), DiGuglielmo disease (C94.0)
- D64.8 Other specified anemias. Childhood pseudoleukemia. Leukoerythroblastic anemia
- D64.9 Anemia, unspecified

Bleeding disorders, purpura and other hemorrhagic conditions (D65 - D69)

D65. Disseminated intravascular coagulation [defibration syndrome]. Afibrinogenemia acquired. Consumptive coagulopathy. Diffuse or disseminated intravascular coagulation (DJC). Acquired fibrinolytic bleeding. Purpura: fibrinolytic, fulminant.
- Excludes: defibration syndrome (complicating): abortion, ectopic or molar pregnancy (O00 - O07, O08.1), in the newborn (P60), pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72 .3)
D66. Hereditary deficiency of factor VIII. Factor VIII deficiency (with functional impairment) Hemophilia: NOS, A, classic.
- Excludes: factor VIII deficiency with vascular disorder (D68.0)
D67. Hereditary deficiency of factor IX. Christmas disease. Deficiency: factor IX (with functional impairment), thromboplastic plasma component, Hemophilia B
D68. Other bleeding disorders.
- Complications excluded: abortion, ectopic or molar pregnancy (O00-O07, O08.1), pregnancy, childbirth and the puerperium (O45.0, O46.0, O67.0, O72.3)
- D68.0 Von Willebrand's disease. Angiohemophilia. Factor VIII deficiency with vascular impairment. Vascular hemophilia.
- Excluded: hereditary capillary fragility (D69.8), factor VIII deficiency: NOS (D66), with functional impairment (D66)
- D68.1 Hereditary factor XI deficiency. Hemophilia C. Deficiency of plasma thromboplastin precursor.
- D68.2 Hereditary deficiency of other coagulation factors. Congenital afibrinogenemia. Deficiency: AC globulin, proaccelerin. Factor deficiency: I (fibrinogen), II (prothrombin), V (labile), VII (stable), X (Stewart-Prower), XII (Hageman), XIII (fibrin-stabilizing). Dysfibrinogenemia (congenital). Hypoproconvertinemia Ovren's disease
- D68.3 Hemorrhagic disorders caused by anticoagulants circulating in the blood. Hyperheparinemia Increased levels of: antithrombin, anti-VIIIa, anti-IXa, anti-Xa, anti-XIa.
- D68.4 Acquired coagulation factor deficiency. Coagulation factor deficiency due to: liver disease, vitamin K deficiency.
- Excludes: vitamin K deficiency in the newborn (P53)
- D68.8 Other specified bleeding disorders. Presence of systemic lupus erythematosus inhibitor
- D68.9 Coagulation disorder, unspecified
D69.Purpura and other hemorrhagic conditions.
- Excluded: benign hypergammaglobulinemic purpura (D89.0), cryoglobulinemic purpura (D89.1), idiopathic (hemorrhagic) thrombocythemia (D47.3), purpura fulminans (D65), thrombotic thrombocytopenic purpura (M31.1)
- D69.0 Allergic purpura. Purpura: anaphylactoid, Henoch (- Schonlein), non-thrombocytopenic: hemorrhagic, idiopathic, vascular. Allergic vasculitis.
- D69.1 Qualitative platelet defects. Bernard-Soulier syndrome (giant platelets), Glanzmann's disease, Gray platelet syndrome, Thrombasthenia (hemorrhagic) (hereditary). Thrombocytopathy.
- Excludes: von Willebrand disease (D68.0)
- D69.2 Other non-thrombocytopenic purpura. Purpura: NOS, senile, simple.
- D69.3 Idiopathic thrombocytopenic purpura. Evans syndrome
- D69.4 Other primary thrombocytopenias.
- Excluded: thrombocytopenia with absent radius (Q87.2), transient neonatal thrombocytopenia (P61.0), Wiskott-Aldrich syndrome (D82.0)
- D69.5 Secondary thrombocytopenia
- D69.6 Thrombocytopenia, unspecified
- D69.8 Other specified hemorrhagic conditions. Capillary fragility (hereditary). Vascular pseudohemophilia.
- D69.9 Hemorrhagic condition, unspecified

Other diseases of the blood and hematopoietic organs (D70 - D77)

D70. Agranulocytosis. Agranulocytic tonsillitis. Children's genetic agranulocytosis. Kostmann's disease. Neutropenia: NOS, congenital, cyclic, drug-induced, periodic, splenic (primary), toxic. Neutropenic splenomegaly. If it is necessary to identify the drug that caused the neutropenia, use an additional code for external causes (class XX).
- Excludes: transient neonatal neutropenia (P61.5)
D71. Functional disorders of polymorphonuclear neutrophils. Defect of the cell membrane receptor complex. Chronic (children's) granulomatosis. Congenital dysphagocytosis. Progressive septic granulomatosis.
D72. Other white blood cell disorders.
- Excluded: basophilia (D75.8), immune disorders (D80 - D89), neutropenia (D70), preleukemia (syndrome) (D46.9)
- D72.0 Genetic abnormalities of leukocytes. Anomaly (granulation) (granulocyte) or syndrome: Alder, May - Hegglin, Pelger - Huet. Hereditary leukocyte: hypersegmentation, hyposegmentation, leukomelanopathy.
- Excludes: Chediak - Higashi syndrome (- Steinbrink) (E70.3)
- D72.1 Eosinophilia. Eosinophilia: allergic, hereditary.
- D72.8 Other specified white blood cell disorders. Leukemoid reaction: lymphocytic, monocyte, myelocytic. Leukocytosis. Lymphocytosis (symptomatic). Lymphopenia. Monocytosis (symptomatic). Plasmacytosis.
- D72.9 White blood cell disorder, unspecified
D73. Spleen diseases
- D73.0 Hyposplenism. Postoperative asplenia. Atrophy of the spleen.
- Excludes: asplenia (congenital) (Q89.0)
- D73.1 Hypersplenism.
- Splenomegaly excluded: NOS (R16.1), congenital (Q89.0).
- D73.2 Chronic congestive splenomegaly
- D73.3 Abscess of the spleen
- D73.4 Splenic cyst
- D73.5 Splenic infarction. Splenic rupture is non-traumatic. Torsion of the spleen.
- Excludes: traumatic splenic rupture (S36.0)
- D73.8 Other diseases of the spleen. Splenic fibrosis NOS. Perisplenitis. Splenitis NOS.
- D73.9 Disease of the spleen, unspecified
D74.Methemoglobinemia
- D74.0 Congenital methemoglobemia. Congenital deficiency of NADH-methemoglobin reductase. Hemoglobinosis M (Hb-M disease). Methemoglobinemia is hereditary
- D74.8 Other methemoglobinemia. Acquired methemoglobinemia (with sulfhemoglobinemia). Toxic methemoglobinemia
- D74.9 Methemoglobinemia, unspecified
D75. Other diseases of the blood and hematopoietic organs.
- Excluded: enlarged lymph nodes (R59.-), hypergammaglobulinemia NOS (D89.2), lymphadenitis; NOS (I88.9), acute (L04.-), chronic (I88.1), mesenteric (acute) (chronic) (I88.0)
- D75.0 Familial erythrocytosis. Polycythemia: benign, familial.
- Excludes: hereditary ovalocytosis (D58.1)
- D75.1 Secondary polycythemia. Polycythemia: acquired, associated with: erythropoietins, decreased plasma volume, altitude, stress, emotional, hypoxemic, nephrogenic, relative.
- Polycythemia excluded: newborn (P61.1), true (D45)
- D75.2 Essential thrombocytosis. Excludes: essential (hemorrhagic) thrombocythemia (D47.3)
- D75.8 Other specified diseases of the blood and hematopoietic organs. Basophilia
- D75.9 Disease of the blood and hematopoietic organs, unspecified
D76. Certain diseases involving lymphoreticular tissue and the reticulohistiocytic system.

- Excluded: Letterer-Siwe disease (C96.0), malignant histiocytosis (C96.1), reticuloendotheliosis or reticulosis: histiocytic medullary (C96.1), leukemic (C91.4), lipomelanotic (I89.8), malignant (C85. 7), non-lipid (C96.0)
- D76.0 Langerhans cell histiocytosis, not elsewhere classified. Eosinophilic granuloma. Hand - Schuller - Christian disease (Histiocytosis X (chronic)
- D76.1 Hemophagocytic lymphohistiocytosis. Familial hemophagocytic reticulosis. Histiocytoses from mononuclear phagocytes other than Langerhans cells, NOS.
- D76.2 Hemophagocytic syndrome associated with infection
- D76.3 Other histiocytosis syndromes. Reticulohistiocytoma (giant cell). Sinus histiocytosis with massive lymphadenopathy. Xanthogranuloma
D77. Other disorders of the blood and hematopoietic organs in diseases classified in other headings. Splenic fibrosis in schistosomiasis [bilharzia] (B65.-)

Selected disorders involving the immune mechanism (D80 - D89)

- Includes: defects in the complement system, immunodeficiency disorders other than human immunodeficiency virus disease HIV, sarcoidosis.
- Excludes: autoimmune diseases (systemic) NOS (M35.9), functional disorders of polymorphonuclear neutrophils (D71), human immunodeficiency virus [HIV] disease (B20 - B24)

D80. Immunodeficiencies with predominant antibody deficiency
- D80.0 Hereditary hypogammaglobulinemia. Autosomal recessive agammaglobulinemia (Swiss type). X-linked agammaglobulinemia [Bruton], (with growth hormone deficiency).
- D80.1 Non-familial hypogammaglobulinemia. Agammaglobulinemia with the presence of B-lymphocytes carrying immunoglobulins. General agammaglobulinemia. Hypogammaglobulinemia NOS
- D80.2 Selective deficiency of immunoglobulin A IgA
- D80.3 Selective deficiency of immunoglobulin G subclasses IgG
- D80.4 Selective deficiency of immunoglobulin M IgM
- D80.5 Immunodeficiency with elevated levels of immunoglobulin M (IgM)
- D80.6 Antibody deficiency with immunoglobulin levels close to normal or with hyperimmunoglobulinemia. Antibody deficiency with hyperimmunoglobulinemia.
- D80.7 Transient hypogammaglobulinemia of children
- D80.8 Other immunodeficiencies with a predominant antibody defect. Kappa light chain deficiency
- D80.9 Immunodeficiency with predominant antibody defect, unspecified
D81. Combined immunodeficiencies.
- Excludes: autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
- D80.0 Severe combined immunodeficiency with reticular dysgenesis
- D80.1 Severe combined immunodeficiency with low T- and B-cell content
- D80.2 Severe combined immunodeficiency with low or normal B cell count
- D80.3 Adenosine deaminase deficiency.
- D80.4 Nezelof syndrome
- D80.5 Purine nucleoside phosphorylase deficiency
- D80.6 Deficiency of class I molecules of the major histocompatibility complex. Bald Lymphocyte Syndrome
- D80.7 Deficiency of class II molecules of the major histocompatibility complex
- D80.8 Other combined immunodeficiencies. Biotin-dependent carboxylase deficiency
- D80.9 Combined immunodeficiency, unspecified. Severe combined immunodeficiency disorder NOS
D82. Immunodeficiencies associated with other significant defects.
- Excludes: ataxia-telangiectasia (Louis-Bar syndrome) (G11.3)
- D82.0 Wiscott-Aldrich syndrome. Immunodeficiency with thrombocytopenia and eczema
- D82.1 Di George syndrome. Pharyngeal diverticulum syndrome. Thymus: alymphoplasia, aplasia or hypoplasia with immune deficiency.
- D82.2 Immunodeficiency with dwarfism due to short limbs
- D82.3 Immunodeficiency due to a hereditary defect caused by the Epstein-Barr virus. X-linked lymphoproliferative disease
- D82.4 Hyperimmunoglobulin E IgE syndrome
- D82.8 Immunodeficiency associated with other specified significant defects
- D82.9 Immunodeficiency associated with significant defect, unspecified
D83. Common variable immunodeficiency
- D83.0 General variable immunodeficiency with predominant abnormalities in the number and functional activity of B cells
- D83.1 General variable immunodeficiency with a predominance of disorders of immunoregulatory T cells
- D83.2 Common variable immunodeficiency with autoantibodies to B or T cells
- D83.8 Other common variable immunodeficiencies.
- D83.9 Common variable immunodeficiency, unspecified
D84. Other immunodeficiencies
- D84.0 Lymphocyte functional antigen-1 defect
- D84.1 Defect in the complement system. Cl esterase inhibitor (C1-INH) deficiency
- D84.8 Other specified immunodeficiency disorders
- D84.9 Immunodeficiency, unspecified
D86.Sarcoidosis
- D86.0 Pulmonary sarcoidosis
- D86.1 Sarcoidosis of lymph nodes
- D86.2 Sarcoidosis of the lungs with sarcoidosis of the lymph nodes
- D86.3 Sarcoidosis of the skin
- D86.8 Sarcoidosis of other specified and combined localizations. Iridocyclitis in sarcoidosis (H22.l), Multiple cranial nerve palsies in sarcoidosis (G53.2), Sarcoid: arthropathy (M14.8), myocarditis (I41.8), myositis (M63.3). Uveoparotitic fever, Herfordt's disease
- D86.9 Sarcoidosis, unspecified
D89. Other disorders involving the immune mechanism, not classified elsewhere.
- Excluded: hyperglobulinemia NOS (R77.1), monoclonal gammopathy (D47.2) graft failure and rejection (T86.-)
- D89.0 Polyclonal hypergammaglobulinemia. Hypergammaglobulinemic purpura. Polyclonal gammopathy NOS
- D89.1 Cryoglobulinemia. Cryoglobulinemia: essential, idiopathic, mixed, primary, secondary, Cryoglobulinemic(s): purpura, vasculitis
- D89.2 Hypergammaglobulinemia, unspecified
- D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified
- D89.9 Disorder involving the immune mechanism, unspecified. Immune disease NOS