Severe blood disease. Diseases of the blood and hematopoietic organs. Clotting diseases - photo gallery

Blood diseases affect the cellular elements of the blood, such as platelets, red blood cells and white blood cells, or the fluid part, i.e. plasma.

Let's look at what these diseases are and analyze the various symptoms that each of them manifests.

Features of blood diseases

When we talk about blood diseases, then we mean certain disorders that affect one or more blood elements.

Blood diseases can affect cellular elements, that is, red blood cells, platelets and other blood cells, as well as its plasma.

Some blood diseases are of genetic origin, others are related to cancer, and others are associated with a deficiency of certain substances. In any case, they can affect people of any category and age, from young children to the elderly.

Classification of hematological diseases

Blood pathologies can be classified depending on the timing of development, the prognosis for the patient’s life, the type of pathology and individual response to therapy, and the type of blood elements affected.

In addition to this, blood diseases are divided depending on the reasons:

The symptomatology of blood diseases differs depending on the affected blood component.

In case of red blood cell disease, symptoms are often associated with oxygen and hemoglobin deficiency:

In case of white blood cell disease Symptoms include:

• enlarged lymph nodes and spleen

Platelet pathologies may manifest themselves:

• patches on the skin that look like bruises, or bleeding for no reason when the platelet count becomes too low
• the formation of blood clots and clots when the platelet count is excessive

In the presence of plasma pathologies may manifest themselves:

• blood clotting difficulties
• bleeding

The most common blood pathologies

Let's now try to list the most common blood diseases. For convenience, we divided the diseases depending on the type of affected blood fraction.

Diseases of red blood cells (erythrocytes)

The “main” disease of red blood cells is called anemia and are characterized by a decrease in the number of circulating red blood cells due to reduced production or accelerated destruction.

Among the anemias, the most famous are:

• Sickle cell anemia: This is a genetically inherited pathology and is characterized by a change in the physical shape of red blood cells, which take on a sickle shape and which break easily.
• Iron-deficiency anemia: This is a type of anemia caused by iron deficiency due to nutritional problems or congenital causes. Iron is not absorbed properly or is supplied in insufficient quantities, so red blood cells and hemoglobin are not formed properly.
• Pernicious anemia: Caused by vitamin B12 deficiency due to poor diet or deficiency of intrinsic factor necessary for the absorption of this vitamin. Due to a lack of vitamin B12, red blood cells do not mature properly.
• Autoimmune hemolytic anemias: Groups together several autoimmune diseases in which the immune system attacks and destroys red blood cells. The cause is often unknown, but may be due to another disease, such as lymphoma, or triggered by medications.
• Aplastic anemia: The disease is characterized by the inability of the bone marrow to properly produce red blood cells and other blood cells. The exact cause of the pathology is unknown, but it is believed to be due to the interaction of genetic and environmental factors.
• Secondary anemia of chronic diseases: This type of anemia appears in patients suffering from chronic diseases such as kidney failure. Since in this case the factor necessary for the proper synthesis and maturation of red blood cells is not produced.
• Thalassemia: This is an inherited disease associated with a gene mutation that determines the development of chronic anemia and is potentially fatal to the patient.
• Hereditary A spherocytosis: This is a genetically inherited disease in which the formation of red blood cell membrane proteins is disrupted. This means that red blood cells can be easily destroyed and, as a result, anemia develops and the spleen enlarges.
• G6DP deficiency: Also known as favism, this is an inherited genetic disorder in which the enzyme glucose-6-phosphate dehydrogenase is not synthesized. This defines the occurrence of hemolytic crises (ie the sudden destruction of red blood cells) as a response to various causes, for example, after ingestion of certain foods, including legumes.

Among other blood diseases not classified as anemia, but which affect red blood cells, we have:

Diseases of white blood cells have mainly tumor pathology, which determines changes in the number of cells of the immune system (mainly leukocytosis, i.e. increase in the number of white cells in the blood).

Among these blood diseases we have:

• Myeloma: a tumor common in older people that affects cells of the immune system. There are different types of myeloma, but most cases present with widespread bone pain and anemia.
• Leukemia: This is an oncological pathology that leads to overproduction of blood cells, in particular, cells of the immune system. There are different types (myeloid, acute, chronic, lymphoid) and mainly affects young people and children, although it can also occur in adults. In some cases it has a genetic origin, but is not hereditary, in other cases it can be determined by exposure to environmental factors.
• Lymphoma: cancer that affects the B and T cell lines of the immune system. There are two main types, non-Hodgkin lymphoma (the most common form) and Hodgkin lymphoma, its development is associated with the influence of infectious diseases and autoimmune diseases.

Among other non-oncological pathologies that affect white blood cells, we can mention:

Pathologies that affect platelets and lead to blood clotting defects, since these cells are involved in blood clotting processes.

Among these diseases we have:

• Thrombocytopenic purpura: This is a disease in which there is a decrease in the number of platelets as a result of autoimmune processes that destroy platelets in the blood. There is an increase in blood clotting time and blood clot formation. The cause is still not clearly defined, but the influence of temporary conditions such as pregnancy and genetic factors has been noted.
• Basic thrombocythemia: In this case, the bone marrow produces an excessive amount of platelets. The reason is also not completely clear, since the pathology is very rare. Leads to excessive clot formation, which can obstruct arteries and veins, causing stroke or heart attack.
• Idiopathic thrombocytopenic purpura: This is an autoimmune pathology in which we see a decrease in the number of platelets (thrombocytopenia). The causes are currently unknown, and the pathology is manifested by severe bleeding.
• Glazman's thrombasthenia: This is a pathology in which platelets lose the ability to aggregate among themselves and interact with fibrinogen to form a blood clot. This is a rare disease caused by the absence or deficiency of a special protein on the surface of platelets. Causes frequent bleeding.

Plasma diseases

Plasma pathologies include all those diseases that are caused by a systemic condition or deficiency of substances present in the blood, for example:

• Hemophilia: a genetic inherited disorder associated with the X chromosome that causes frequent bleeding due to the absence of one of the blood clotting factors normally present in blood plasma: factor 8(for hemophilia type A) or factor 9(for hemophilia type B). The consequence of this is the inability of blood to clot properly, so even a small superficial wound can lead to fatal bleeding.
• von Willebrand disease: This is a pathology associated with a deficiency of von Willebrand factor, which is manifested by bleeding and the inability of the blood to clot properly. Exists in three different forms. This is a genetic disease caused by a change on chromosome 12.
• Widespread intravascular coagulation: This is a very dangerous disease and often fatal, as it causes the formation of blood clots in various vessels, leading to ischemic damage to organs and tissues. It develops due to massive activation of blood clotting factors due to various reasons, such as poisoning, tumors and infections.
• Autoimmune diseases: such as rheumatoid arthritis and lupus erythematosus, which is caused by the presence in the blood plasma of antibodies against joint cells, in the first case, or against various organs and tissues, in the second case.
• Blood poisoning: mainly affects older people and people with weakened immune systems. This is a condition characterized by an infectious process in the blood, which then spreads to all organs and tissues. In this case, the bacteria are localized at the level of blood plasma, as a result of which the infection becomes systemic.

Regular medical examinations are very important for the early detection of blood diseases and defeat even the most aggressive ones!

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Blood diseases constitute a large and heterogeneous group of syndromes that develop when there are disturbances in the qualitative and quantitative composition of the blood. The practical direction that develops the principles of diagnosis and treatment of blood diseases is hematology and its separate branch - oncohematology. Specialists who correct the condition of the blood and hematopoietic organs are called hematologists. Hematology has the closest interdisciplinary ties with internal medicine, immunology, oncology, and transfusiology.

Since ancient times, in many cultures, human blood has been endowed with mystical properties, symbolizing the divine source and flow of life. “Precious”, “hot”, “innocent”, “young”, “royal” - whatever properties people endowed with blood, and the epithet “blood” always meant the highest degree of certain manifestations - consanguinity, blood enemy, blood resentment , blood feud.

Meanwhile, from the point of view of physiology, blood is a liquid medium of the body, continuously circulating through the vascular system and performing a number of important functions - respiratory, transport, regulatory, protective, etc. Blood consists of a liquid fraction - plasma and formed elements suspended in it - blood cells (erythrocytes, platelets and leukocytes). The main organs of hematopoiesis (hemocytopoiesis), which are a kind of “factory” for the production of blood cells, include red bone marrow, thymus, lymphoid tissue and spleen. Blood diseases are spoken of when there is a violation of the morphology or number of certain blood cells or changes in the properties of plasma.

All diseases of the blood and hematopoietic system are classified based on the damage to one or another of its components. In hematology, blood diseases are usually divided into three large groups: anemia, hemoblastosis and hemostasiopathy. Thus, frequent anomalies and lesions of erythrocytes include deficiency, hemolytic, hypo- and aplastic anemia. The structure of hemoblastoses includes leukemia and hematosarcoma. Blood diseases associated with damage to the hemostatic system (hemostasiopathy) include hemophilia, von Willebrand disease, thrombocytopathies, thrombocytopenia, disseminated intravascular coagulation syndrome, etc.

Blood diseases can be congenital or acquired. Congenital diseases (sickle cell anemia, thalassemia, hemophilia, etc.) are associated with gene mutations or chromosomal abnormalities. The development of acquired blood diseases can be triggered by numerous environmental factors: acute and chronic blood loss, exposure of the body to ionizing radiation or chemical agents, viral infections (rubella, measles, mumps, influenza, infectious mononucleosis, typhoid fever, viral hepatitis), nutritional deficiency, impaired absorption of nutrients and vitamins in the intestines, etc. When bacterial or fungal agents penetrate into the blood, a serious blood disease of infectious origin occurs - sepsis. Many blood diseases go hand in hand with collagenoses.

Manifestations of blood diseases are many-sided and not always specific. Characteristic signs of anemia include unreasonable fatigue and weakness, dizziness and even fainting, shortness of breath during exercise, and pale skin. Blood coagulation disorders are characterized by petechial hemorrhages and ecchymosis, various types of bleeding (gingival, nasal, uterine, gastrointestinal, pulmonary, etc.). In the clinic of leukemia, intoxication or hemorrhagic syndromes come to the fore.

The very first method with which the diagnosis of blood diseases begins is the study of a hemogram (clinical analysis) with determination of the quantitative composition of the blood and the morphology of the formed elements. For blood diseases that occur with impaired hemostasis, the number of platelets, blood clotting and bleeding time, prothrombin index, coagulogram are examined; Various types of tests are carried out - a tourniquet test, a pinch test, a jar test, etc.

To assess changes occurring in the hematopoietic organs, bone marrow puncture (sternal puncture) is used. To determine the causes of anemic syndrome, patients may need to consult a gastroenterologist and gynecologist; an FGDS, colonoscopy, and liver ultrasound.

Any changes in hemogram or myelogram, as well as symptoms indicating the likelihood of developing blood diseases, require a competent assessment by a hematologist, dynamic observation or specialized treatment under his supervision. Modern hematology has developed the fundamental principles of treatment for various blood diseases and has accumulated vast experience in curing them. If possible, treatment of blood diseases begins with eliminating the causes and risk factors, correcting the functioning of internal organs, replenishing missing substances and microelements (iron - for iron deficiency anemia, vitamin B12 - for B12-deficiency anemia, folic acid - for folate deficiency anemia, etc. .).

In some cases, taking corticosteroids, hemostatic drugs, and extracorporeal hemocorrection (erythrocytapheresis) may be indicated. Hematological patients often require transfusions of blood and its components. The most relevant and effective methods for treating hematoblastoses around the world today are cytostatic therapy, radiotherapy, allogeneic and autologous bone marrow transplantation, and the introduction of stem cells. A number of blood diseases (thrombocytopenic purpura, autoimmune anemia, myeloid leukemia, etc.) are an indication for removal of the spleen - splenectomy. Treatment of blood diseases in Moscow is carried out in specialized hematological medical and scientific centers with world-class technical equipment and highly qualified specialists.

» you will be able to find answers to basic questions regarding blood diseases, get acquainted with the main nosological forms, symptoms, principles of diagnosis and treatment.

Blood diseases are associated with various disturbances in the number of formed elements, their structure, size or properties of plasma. In medicine, the term “systemic blood diseases” is more commonly accepted. It represents a broader concept and includes the pathology of organs that produce red blood cells, platelets and leukocytes (bone marrow, lymph nodes and spleen).

Population Prevalence

In terms of the number of patients, blood diseases are not included in the “15 diseases” defined by the World Health Organization as the most common in the world.

But in Russia, statistics indicate very disappointing figures: compared to 1990, the incidence of blood system diseases in the population has increased 3.6 times.

This has caused the need to develop hematological research, search for means to combat pathology, further expand the number of specialized beds in specialized hospitals, and train doctors of all specialties.

Primary care physicians are required to know what clinical manifestations are associated with pathologies of the blood and hematopoietic organs in order to promptly refer the patient to a hematologist.

The lower blue line shows a graph of the growth of blood diseases by year

Causes

Many reasons leading to changes in the blood have been studied. But even more mysteries remain. Scientists believe that they have the right to point out risk factors in such cases.

• acute and chronic blood loss lead to disturbances in blood formation and increased cell breakdown in anemia;
• mutations of the human genome under the influence of chemicals (cytostatics, antibiotics, industrial poisons), radioactive radiation;
• hereditary transmission of predisposition to the disease occurs in families where blood diseases occur in children;
• HIV infection and Epstein-Barr virus.

It is impossible to unambiguously associate any of the reasons with the disease of a particular person. Only a combination of disadvantages in life situations can lead to illness.

Classifications

The International Classification of Diseases (ICD-10) identifies more than 90 nosological units for blood diseases.

A simplified clinical classification identifies 4 groups of diseases associated with one leading pathological process:

• anemia - defined as a condition with a reduced level of hemoglobin;
• hemorrhagic diathesis - all diseases with impaired coagulation;
• hemoblastosis - tumor diseases of cell sprouts from the bone marrow and lymph nodes;
• a group of other pathological manifestations.

Each group is further subdivided into many subtypes, depending on the clinical course, damage to specific blast cells, and the leading causes of the disease.

For information about blood diseases, it is important to take into account the primary signs of the disease. Therefore, in practice, a syndromic classification with the identification of leading symptom complexes is more suitable. All symptoms in it can be divided into general and local, which characterize lesions of a specific hematopoietic organ. Many diseases have the same patient complaints and similar clinical manifestations.

Diseases with anemic syndrome

The group includes all types of anemia associated with a decrease in hemoglobin content, impaired transport and absorption of oxygen and the development of tissue hypoxia (oxygen starvation).

Symptoms:

• persistent headaches;
• dizziness, fainting;
• sensation of “tinnitus”;
• dyspnea;
• tachycardia and stabbing pain in the heart area;
• “darkening or flickering spots” in the eyes;
• increased fatigue;
• decreased memory;
• irritability, insomnia.

In older people, even with a moderate decrease in hemoglobin, heart failure occurs.

Specific symptoms of a particular type of anemia are not included here (perverted taste in iron deficiency anemia, yellowness of the skin and sclera in hemolytic or hemorrhage, in hypoplastic anemia).

Diseases with manifestation of ulcerative-necrotic changes

Ulcerative-necrotic lesions are possible with a significant decrease or disappearance of the granulocytic lineage of the blood, leukemia.
Symptoms:

• sore throat;
• impaired swallowing process due to severe pain;
• salivation;
• attacks of abdominal pain, bloating;
• diarrhea;
• smell from the mouth;
• pain in the anus.

Upon examination, ulcers are found in the oral cavity (stomatitis), on the mucous membrane of the pharynx (angina), during esophagoscopy - in the esophagus, and during colonoscopy - in the intestines.

This is what stomatitis looks like

Diseases with hemorrhagic changes

The syndrome is caused by a decrease in the total number of platelets or their impaired function, increased permeability of the vascular wall, and increased consumption of fibrinogen and platelets during the coagulation process. Characteristic of thrombocytopenic purpura, hemophilia, aplastic anemia, hemorrhagic vasculitis.

Symptoms:

• hemorrhages in the skin, mucous membranes (sclera of the eyes), muscles, joints, injection sites;
• bleeding and bleeding from the nose, gums, intestines, heavy menstruation.

Diseases with enlarged lymph nodes

The syndrome is called lymphadenopathic. Accompanied by the proliferation of lymph node tissue, compression of neighboring vessels and organs. Depending on the location of the enlarged nodes, symptoms appear:

• shortness of breath and dry cough (mediastinal lymph nodes);
• “overflowing” of the intestines, bloating, stool disorders (retroperitoneal and mesenteric nodes).

This is what enlarged lymph nodes in the neck look like

Diseases with increased body temperature

The causes of fever in blood pathologies lie in the specific pyrogenic effect of the breakdown products of blood cells and tissues during the ulcerative process. Develops with hemoblastosis, lymphosarcoma.

Symptoms: prolonged increase in body temperature, sweating, chills.

Diseases with severe intoxication

Intoxication manifests itself in different ways in blood pathologies:

• general weakness - in any form caused by anemia and cell destruction (anemia, leukemia);
• itching of the skin - due to the release of histamine from blood cells, increased levels of basophils (lymphogranulomatosis, myeloid leukemia), impaired microcirculation in the skin (erythremia);
• lack of appetite, weight loss - with malignant lymphomas.

Symptoms of fever and intoxication in some diseases appear during the day, in others - at night

Diseases with pain in joints and bones

Such a symptom as osteoarthropathic syndrome is associated with inflammation of the joints, hemorrhage into the joint capsule, and the growth of tumor tissue into bone tissue.

Symptoms:

• pain in the spine, ribs, iliac bones, skull, less often in the tubular bones of the extremities (myeloma), often independent of anything, intensified by beating;
• radicular radiculitis pain (with lymphogranulomatosis, multiple myeloma) due to germination into nerve trunks;
• joint pain (hemolytic anemia, lymphogranulomatosis, hemophilia);
• swelling and redness of the joints, dysfunction.

Immunodeficiency diseases

A decrease in immunity occurs when there is a deficiency of cells, the production of antibodies to one’s own tissues. The condition is manifested by frequent colds, complicated by infections, pneumonia, and bronchitis. Any small wounds on the skin lead to suppuration. In the kidneys, inflammation causes pyelonephritis, and an autoimmune process causes glomerulonephritis.

Diseases with impaired plasma protein levels

Symptoms:

• frequent headaches;
• memory loss;
• numbness and pain in the limbs;
• increased bleeding of the gums, tongue, nose;
• increased blood pressure;
• decreased vision.

Diseases with enlargement of the liver and spleen

The pathology is called hepato-splenomegaly. Develops in infectious mononucleosis, autoimmune hemolytic, sickle cell and B 12 deficiency anemia, thrombocytopenia, acute leukemia, chronic lympho- and myeloid leukemia.

Characterized by symptoms:

• heaviness or pain in the upper abdomen;
• abdominal enlargement;
• increasing weakness;
• in later stages, yellowness of the skin.

Other syndromes are less common. Sometimes a blood reaction is caused by the presence of a chronic disease. To identify this at the first manifestations, the patient is asked to undergo a full examination.

How to prevent blood diseases

For prevention, you need to try to avoid all harmful factors:

• treat any diseases and conditions accompanied by bleeding;
• take seriously the manifestations and treatment of helminthic infestations in children and adults;
• complete treatment of acute infections;
• Take enough vitamins and minerals with food every day;
• control the radiation dose during x-ray examination, avoid staying near radiation sources;
• reduce contact, use mandatory protective equipment when working with paints, benzene, lead salts, pesticides and other hazardous substances;
• strengthen immunity;
• do not expose yourself to hypothermia or overheating;
• learn to cope with stressful situations with minimal losses.

These measures normalize the process of hematopoiesis and help maintain health and ability to work.

11-02-2012, 19:47

Description

Anemia

Anemia, or anemia, is a condition characterized by a decrease in the number of red blood cells and a decrease in the hemoglobin content per unit volume of blood. In some cases, with anemia, qualitative changes in red blood cells are also detected.

In anemia, as a result of transport dysfunction, hypoxia phenomena, the signs of which are shortness of breath, tachycardia, discomfort in the heart, dizziness, weakness, fatigue, pallor of the skin and visible mucous membranes. The severity of these symptoms depends on the degree of anemia and the speed of its development. In case of deep anemia, along with the indicated symptoms, visual impairment.

By color indicator anemia is divided into hypochromic, normochromic and hyperchromic. Based on the average diameter of erythrocytes, anemia is divided into microcytic, normocytic and macrocytic. Based on the nature of regeneration, anemia is distinguished as regenerative, hyporegenerative, hypo- and aplastic, dysplastic or dyserythropoietic.

Currently generally accepted classification, built on a pathogenetic principle, taking into account the etiological and most important clinical and morphological forms, is the classification proposed by G. A. Alekseev (1970).

I. Anemia due to blood loss (posthemorrhagic).
II. Anemia due to poor circulation:
A. Iron deficiency anemia (“chloranemia”).
B. Iron-saturated, sideroachrestic anemia.
B. B12 (folate) deficiency, “pernicious” anemia:
1. Exogenous deficiency of vitamin B12 (folic acid).
2. Endogenous deficiency of vitamin B12 (folic acid):
a) impaired assimilation of dietary vitamin B12 due to loss of gastric mucoprotein secretion;
b) impaired assimilation of vitamin B12 (folic acid) in the intestines;
c) increased consumption of vitamin B12 (folic acid).
G. B12 (folate) - “achrestic” anemia.
D. Hypoaplastic anemia:
1. Due to the influence of exogenous factors.
2. Due to endogenous bone marrow aplasia.
E. Metaplastic anemia.
III. Anemia due to increased blood destruction (hemolytic):
A. Anemia caused by exoerythrocyte hemolytic factors.
B. Anemia caused by endoerythrocyte factors:
1. Erythrocytopathies.
2. Enzymopenia:
a) deficiency of glucose-6-phosphate dehydrogenase;
b) pyruvate kinase deficiency;
c) deficiency of glutathione reductase.
3. Hemoglobinopathies.

Below we describe the characteristic features of individual forms of anemia, in which eye symptoms are most common.

Acute posthemorrhagic anemia develops as a result of acute single and repeated blood loss from injuries, bleeding from the gastrointestinal tract, ectopic pregnancy, uterine bleeding, etc. Symptoms of the disease are pathogenetically associated with a decrease in the mass of circulating blood and oxygen deficiency. The clinical picture in the first moments after massive blood loss fits into the clinic of post-hemorrhagic shock or collapse: pale skin, fainting, dizziness, cold sweat, rapid thready pulse, sometimes vomiting, convulsions. Subsequently, as the general condition improves and blood pressure stabilizes, the symptoms of anemia and hypoxia begin to predominate in the clinical picture. It is during this period that signs of visual impairment, up to complete amaurosis, are most often detected, since specific elements of the retina are very sensitive to anemia.

For chronic hypochromic iron deficiency anemia, including early and late chlorosis, symptomatic iron deficiency anemia (chronic enteritis, agastric chloranemia, hiatal hernia, malignant neoplasms, chronic infections), as well as chronic hypochromic megaloblastic anemia (pernicious anemia of various origins - Addison-Birmer anemia, helminthic, spruanemia , celiac disease, etc.) the severity of eye symptoms depends on the degree of anemia, which, however, varies widely individually. Changes in the fundus occur especially often when the hemoglobin concentration is below 5 g% and less often 7 g%.

Ocular fundus with anemia he looks pale. This symptom cannot always be assessed due to differences in pigmentation of the retina and choroid. Decoloration of the optic nerve head and retinal vessels is easier to detect. In this case, the arterial vessels tend to expand and approach the caliber of similar venous branches. Multiple hemorrhages into the retina - the most characteristic symptom of retinopathy in anemia (Fig. 34).

Rice. 34. Fundus of the eye in pernicious anemia.

The cause of the hemorrhages is not entirely clear. Apparently lack of oxygen causes increased capillary permeability. In pernicious anemia, concomitant thrombocytopenia is also important.

Striped or flame shaped hemorrhages are located in the layer of nerve fibers. They can be localized in any part of the retina, but they are not in the macula. Therefore, visual acuity is usually preserved. Sometimes a white center is visible in extravasates. This symptom is more often observed in pernicious anemia. In some cases, ischemia may cause swelling of the optic disc and adjacent retina. Usually the swelling is mild, but cases of stagnant disc have also been described. In addition to edema, there may be small white lesions in the layer of nerve fibers, which consist of fibrin and usually resolve well when the patient’s condition improves.

Significantly more severe retinal changes are observed with sickle cell (drepanocytic) anemia. This disease belongs to hereditary familial hemolytic anemia, a characteristic feature of which is the ability of red blood cells to take a sickle shape - this disease affects mainly blacks and rarely white people. Isolated cases have been described in the Soviet Union.

The disease belongs to the group hemoglobinopathies with congenital inferiority of red blood cells, in particular with the presence of pathological globulin in them.

The disease manifests itself already in childhood and is characterized by a chronic course with frequent exacerbations in the form of hemolytic aregenerative, thrombotic and sequestral crises.

During hemolytic crises the content of erythrocytes can drop to 1-2 million in 1 mm3 of blood over a short period. The crisis is accompanied by the development of jaundice and abdominal syndrome. Regenerative crises are temporary, functional depletion of bone marrow hematopoiesis. Thrombotic or painful crises, which sometimes dominate the manifestations of the disease, occur due to generalized thrombosis of small vessels, especially the abdominal cavity and extremities. Sequestral crises are conditions resembling shock with the sudden development of anemia without hemolysis [Tokarev Yu. N., 1966].

As with other congenital hemolytic anemias, patients with sickle cell anemia are infantile, suffer from hypogonadism, have a tower skull, etc. With this disease: the osteoarticular syndrome is especially pronounced (dactyllitis, pain, deformation, necrosis of the articular heads and bones). Chronic ulcers often develop on the legs. The spleen and liver are enlarged. Thrombosis and embolism are a very characteristic sign. Retinal lesions are localized mainly in the equatorial and peripheral zones and go through 5 stages. Stage I is characterized by peripheral arteriolar obstruction, stage II - the appearance of arteriovenous anastomoses. In stage III, neovascular and fibrous proliferation develops, which in stage IV leads to hemorrhages into the vitreous body. Ultimately (stage V), retinal detachment develops.

Leukemia

Leukemia is understood as neoplastic diseases, the tumor mass of which consists of blood cells or, what is apparently more accurate, of cells similar in appearance to blood cells.

Some scientists blood tumors are divided on hemoblastomas and hematosarcomas on the basis that in some cases the bone marrow can be ubiquitously populated by these tumor cells, and in other cases their growth occurs extramedullary. In our opinion, such a division is often very difficult to carry out, since tumor growths of leukemic cells can also have extramedullary localization in patients in whom the disease began with bone marrow damage. And, conversely, in some cases of hematosarcomas, the bone marrow may subsequently become involved in the process, and clinicians are forced in these cases to talk about leukemia of the process. In our opinion, it is more correct to unite all tumors of hematopoietic tissue under the name “leukemia,” since the neoplastic nature of these diseases, emphasized in the names “hemoblastosis” or “hematosarcomatosis,” is practically beyond doubt.

Etiology of leukemia cannot be considered definitively clarified, which applies, however, to other tumors equally. However, it can now be considered established that factors such as a virus, ionizing radiation, certain chemical substances, including some medicinal substances such as chloramphenicol, butadione and cytostatics, can have a certain stimulating effect on the occurrence of these diseases. There are also well-founded opinions about the role of hereditary factors in the occurrence of leukemia. They are confirmed by cases of the occurrence of leukemia of the same type in identical twins, a high susceptibility to the development of leukemia in patients with hereditary disorders of the genetic apparatus - Down's disease, Turner syndrome, ... Klinefelter syndrome, etc. It is noted that certain types of leukemia tend to be combined with certain types genetic disorders. It must be borne in mind that modern scientific data are very convincing in favor of the previously put forward assumption about the origin of the entire leukemic mass from one mutated cell that went beyond the control of the patient’s body. These are the presence of a ring chromosome in tumor cells of patients with acute leukemia that developed in individuals treated with radioactive phosphorus, a sharp increase in the content of a protein of the same type in terms of physicochemical properties in patients with paraproteinemic hemoblastoses. Philadelphia chromosome in patients with chronic myeloid leukemia.

In clinical practice Leukemias are usually divided depending on the type of cell that forms the basis of the tumor mass. Those leukemias that occur with the proliferation of poorly differentiated cells and not capable of further differentiation are usually very malignant without treatment and are called acute. Leukemias, the tumor mass of which consists of differentiating and mature cells, usually have a relatively benign course and are called chronic leukemias.

Acute and chronic leukemias in turn are subdivided depending on which cell constitutes the substrate of the tumor. Currently, leukemias are described that develop from cells of all hematopoietic lineages - erythroid, platelet, etc. granulocytic and agranulocytic type. In this case, acute leukemias are distinguished between myelo-, mono-, megakaryo-, erythro- and plasmablastic types. Since differentiation of acute leukemia is carried out only on the basis of cytochemical research methods, and cytochemical methods of cell identification are carried out using an empirically selected set of methods, reports have appeared about the existence of such a form of acute leukemia as undifferentiated. The origin of the latter can apparently be attributed to the proliferation of cells derived from earlier, undifferentiated hematopoietic cells. Among chronic leukemia, forms of leukemia have been identified and continue to be identified, which are based on the proliferation of any mature blood cell. Here are chronic lymphocytic leukemia, chronic myeloid leukemia, chronic monocytic leukemia, chronic megakaryocytic leukemia, erythromyelosis, erythremia, plasmacytoma, chronic basophilic cell leukemia; There are also reports of the presence of chronic eosinophilic leukemia.

With the modern level of medical science, which makes it possible to distinguish the finest details of cells, divisions are made within the framework of seemingly long-established forms of leukemia. Thus, among a group of patients with chronic lymphocytic leukemia Currently, groups of people suffering from proliferation of both T- and B-lymphocytes are already identified, and among patients chronic myeloid leukemia distinguish between groups with proliferation of cells that have the Philadelphia chromosome and those that do not. It is possible that the identification of leukemia will continue in the future, and this will allow for more specific and more effective treatment of patients.

Based on the above, it is quite easy to talk about diagnosing both leukemia itself and its specific form. Diagnosis of this disease is carried out when hyperplasia of hematopoietic tissue is detected, which can occur both in the peripheral blood and in the bone marrow. Moreover, in some individuals, hyperplasia of leukemic cells occurs only in the bone marrow, and in the peripheral blood these cells appear only at later stages of the disease. In this regard, studies of bone marrow hematopoiesis using analysis of sternal puncture data, and sometimes the structure of bone tissue using trepanobiopsy, should be carried out during the diagnostic process. The use of cytochemical and cytogenetic research methods usually only leads to a clarification of the type of leukemia.

Possibility of existence leukemoid reactions, i.e., such growths of hematopoietic tissue that occur in response to the presence in the patient’s body of some factor that activates hematopoiesis, sometimes makes it necessary to conduct special studies that exclude the presence of these causes of hyperplasia of hematopoietic tissue.

Clinical picture leukemia is very diverse. At the same time, a patient with both acute and chronic leukemia has a variety of clinical manifestations. Apparently, not a single experienced clinician will dare to predict the further clinical course and clinical manifestations of leukemia in an individual patient. It is almost impossible to do this due to the fact that the high morphodynamicity and almost universal possible distribution of leukemic tissue in the patient’s body can demonstrate a wide variety of symptoms, simulating, especially in the initial stages, diseases of a very different nature. An example of this is the work of one of the founders of Russian hematology, Acad. I. A. Kassirsky, who, together with his colleagues, when analyzing the primary diagnoses with which patients were admitted to the clinic and in whom acute leukemia was subsequently verified, discovered more than 60 different nosological forms, among which were sepsis, stomach cancer, rheumatism and acute intestinal obstruction, myocardial infarction, rheumatoid arthritis, acute meningitis and many other diseases.

At the same time, it is possible to talk about the clinic of leukemia quite simply due to the fact that all the clinical manifestations of these diseases can be combined and understood on the basis of recognizing the main syndromes, which usually occur in the clinical picture with one or another predominance depending on the type of leukemia diseases. Among these syndromes the most common are the following: 1) general toxic syndrome (or intoxication); its manifestations are fever, weakness, sweating, weight loss, lack of appetite, etc.; 2) hemorrhagic syndrome. Its manifestations are extremely varied, including menorrhagia, skin hemorrhages and cerebral hemorrhages; 3) syndrome of toxic-necrotic lesions of the mucous membranes of the gastrointestinal tract; 4) anemic syndrome; 5) tumor growth syndrome, characterized by the proliferation of leukemic tissue in the body. This also includes enlargement of the lymph nodes, liver, spleen, dysfunction of internal organs due to their compression or violation of the integrity of the growing leukemic tissue.

In addition to the manifestations of these syndromes, which are characteristic of all leukemias, certain types of leukemias, in particular paraproteinemic hemoblastoses(plasmocytoma, Waldenström disease, heavy and light chain diseases), erythremia, have a number of features in the clinical picture that will be described in separate sections. The clinical picture of leukemia (lymphatic type) can sometimes be given a special color by autoimmune reactions, manifested by hemolytic anemia, fever, skin changes, etc.

Without dwelling on the external manifestations of each of the syndromes listed above, I would like to note that in recent years, in the clinical picture of leukemia, manifestations have begun to be noted that can be explained as cytostatic therapy, and by prolonging the life span of patients with this pathology. These include an increase in infectious complications, which are the cause of death in almost 40% of patients with chronic lymphocytic leukemia, an increase in neurological symptoms (especially in patients with acute leukemia, called goat neuroleukemia), as well as the frequent development of uric acid nephropathy in patients with leukemia with symptoms of kidney stones.

Thus, the clinical picture of leukemia can be characterized by with a wide variety of symptoms, which is a consequence of a diverse combination of the above syndromes. Of course, with certain types of leukemia, one can note the predominance of one or another syndrome from those listed above, however, no clinician can underestimate the possibility of including any of them in the clinical chart for any type of leukemia.

Speaking about leukemia, one cannot fail to mention the great progress that has been made by modern medicine in the treatment of these diseases. After all, it is with this type of tumor that results have been obtained that allow us to talk about a fundamental cure of a person from a malignant neoplastic disease. The cure of patients with acute lymphoblastic leukemia and lymphogranulomatosis allows us to hope that these successes will extend to the treatment of other forms of leukemia.

Acute and chronic forms of leukemia are accompanied by the same ocular manifestations caused by increased blood viscosity, hypoxia and leukemic infiltration of tissue. These changes include the formation of microaneurysms in the retinal vessels, hemorrhages, cellular infiltration of the choroid, retina, optic nerve and periorbital structures. Infiltration of the meninges can lead to paralysis of the extraocular muscles and the development of a congestive disc. Infiltration of the eyelids, conjunctiva, and orbital tissue with the development of exophthalmos has also been described.

On ophthalmoscopy it is noted pale fundus background. The retinal veins are dilated, tortuous, and whitish stripes are often visible in the retina along their course, representing perivascular leukemic infiltration. Arteries are changed significantly less than veins.

The size and shape of hemorrhages varies. They can be deep, superficial or even preretinal. It is not uncommon to see a white area at the center of a retinal hemorrhage, caused by an accumulation of white blood cells. In the most severe cases, ischemic cotton wool-like lesions appear in the nerve fiber layer, severe swelling of the optic disc and peripapillary retina, and newly formed retinal vessels.

Changes in the fundus in leukemia they occur in approximately 70% of cases, especially often in acute forms. The severity of the changes more or less correlates with the severity of the disease, and with effective treatment of the underlying disease, the condition of the fundus also improves.

Polycythemia

The term "polycythemia" includes group of diseases, which are manifested by an increase in the mass of red blood cells in the body, i.e., an increase in their volume per 1 kg of body weight. The number of red blood cells in 1 mm3 of blood with polycythemia increases to 7-10 million, and the hemoglobin content to 180-240 g/l. There are “true” polycythemia (erythremia, Vaquez disease) and secondary (symptomatic) erythrocytoses.

Erythremia- primary myeloproliferative hematopoietic system disease, which is based on total hyperplasia of the cellular elements of the bone marrow, especially its visual sprout. Therefore, an increased content of leukocytes (up to 9000-15,000 million in 1 mm3 of blood) and platelets (up to 1 million or more) in the blood, along with a more noticeable increase in the number of erythrocytes, is a very characteristic sign of erythremia. G. F. Stroebe (1951) identified three hematological variants of erythremia: 1) without a significant increase in the number of leukocytes and changes in the blood formula; 2) with moderate leukocytosis, neutrophplesis and band shift; 3) with high leukocytosis, neutrophilia and a shift in the blood count to myelocytes. With “true” polycythemia, signs of myelo fibrosis and osteomyelosclerosis with myeloid metaplasia of the spleen are found. As with other myeloproliferative diseases, increased concentrations of alkaline phosphatase, uric acid and vitamin B12 are often found in the blood serum of patients with polycythemia. The clinical picture of polycythemia vera varies depending on the phase of the disease and the severity of the course.

In the advanced, actually erythremic phase of the disease characteristic symptoms are: 1) change in color of the skin and visible mucous membranes; 2) enlargement of the spleen and liver; 3) increased blood pressure; 4) thrombosis and hemorrhage.

Skin changes in the vast majority of patients. They acquire a red-cyanotic hue. The color of the cheeks, tips of the ears, lips and palms changes especially clearly. We emphasize that the color of the skin is dominated by a red tone, but not bright, but cherry. The visible mucous membranes of the lips, tongue and soft palate also acquire a similar shade. The scleral vessels are noticeably injected (rabbit eye sign). Telangiectasias are often found on the cheeks, lips, and tip of the nose, especially in women.

A very characteristic symptom of erythremia is splenomegaly, which is associated with myeloma metaplasia and increased blood supply. Patients with polycythemia vera usually the liver is also enlarged. An increase in its size is also associated with increased blood supply, myeloid metaplasia, proliferation of connective tissue up to the development of cirrhosis or thrombosis of the intrahepatic veins (Budd-Chiari syndrome). In a number of patients, the course of the disease is complicated by the development of cholelithiasis and chronic cholecystohepatitis. The development of these complications is caused by bile plenochromia, which is characteristic of patients with erythremia.

Almost in half of patients with erythremia hypertension is detected, the pathogenesis of which is considered in terms of the body’s compensatory reaction in response to a decrease in stroke and minute volume of blood, an increase in its viscosity and an increase in peripheral resistance (A. V. Demidova, E. M. Shcherbak). The combination of high blood pressure with an enlarged spleen is a cardinal sign of polycythemia vera. If at the same time the patient’s erythrocyte mass increases, then the diagnosis of polycythemia becomes undoubted.

Characterized by paradoxical the tendency of patients with polycythemia and to thrombosis (large arterial and venous vessels of the brain, heart, liver and spleen, small vessels of the hands and feet) and increased bleeding (from stomach and duodenal ulcers, after extraction of teeth, skin hemorrhages and bleeding from the mucous membranes). The cause of bleeding in polycythemia vera is an increase in the mass of circulating blood with overflow of blood vessels and paretic expansion of capillaries, as well as a deficiency of plasma coagulation factors, in particular fibrinogen [Machabeli M. S., 1962], serotonin [Matveenko JI. A., 1965].

Development of thrombosis in erythremia associated with an increase in blood viscosity, a slowdown in blood flow, an increase in the number of platelets and red blood cells, with sclerotic damage to the walls of blood vessels, and general blood hypercoagulability.
In patients with erythremia, the kidneys are often affected (infarctions develop in them due to vascular thrombosis or nephrolithiasis as a result of impaired purine metabolism, which is characteristic of myeloproliferative diseases).

Polycythemia vera characterized by a long course which can be mild, moderate or severe. There are three periods, or phases, in the development of the disease. The first phase of the disease over a long period can occur latently or with mild clinical symptoms. In the early stages, the disease is often mistaken for hypertension.

The clinical picture described above characterizes the developed second, so-called erythremic phase. And in this phase, the course of the disease can be varied.

The terminal phase is characterized by the development of secondary pseudofibrosis with anemia and the disappearance of external signs of erythremia or the development of acute hemocytoblastosis, less commonly, reticulosis.

Unlike polycythemia vera, secondary erythrocytoses are not independent nosological units, but only symptoms of other diseases. The increase in the number of red blood cells and hemoglobin is associated not with the proliferative process in the bone marrow, but with its functional irritation (absolute erythrocytosis) or with blood thickening without increasing erythropoiesis (relative erythrocytosis). The classification below indicates the main types of secondary erythrocytosis, variants of their course, the main pathogenetic mechanisms underlying their development, and specific diseases accompanied by the development of secondary erythrocytosis.

The most noticeable symptom of polycythemia is plethora facial and conjunctiva. Conjunctival and episcleral vessels, especially veins, are dilated, tortuous, and deep red in color. The vessels of the retina have the same appearance (Fig. 35).

Rice. 35. Fundus of the eye in polycythemia.

Attracts attention dark red fundus color. The optic disc is also unusually red in color. You can often see more or less pronounced swelling of the optic disc and peripapillary retina and isolated hemorrhages.

In some cases it develops central retinal vein occlusion. The occlusion appears to be incomplete. The prognosis in such cases is usually favorable, at least significantly better than with occlusion of the central retinal vein of another etiology.

Paraproteinemia

This group of diseases includes primarily multiple myeloma(plasma cell paraproteinemic reticulosis or Rustitsky disease) and macroglobulin reticulolymphomatosis(Waldenström's disease, or macroglobulinemic purpura).

Myeloma is a systemic blood disease of the tumor-hyperplastic type with malignant proliferation of reticuloplasmic type cells. This is leukemia-reticulosis, in particular plasma cell para-(or patho-) proteinemic reticulosis.

Depending on the predominant type of cells, they are distinguished three types of myeloma: 1) reticuloplasmacytoma, 2) plasmablastoma and 3) plasmacytoma.

Proteinuria- a very common symptom of multiple myeloma. As a rule, a micromolecular protein (Bence Jones protein) is excreted in the urine. Proteinuria is associated with the development of myeloma nephropathy - paraproteinemic nephrosis, which usually ends in death due to symptoms of azotemic uremia.

A high concentration of protein in the blood is also associated with myeloma. high blood viscosity.

Waldenström's disease is currently considered as macroglobulin reticulolymphomatosis, the characteristic feature of which is the ability synthesize macroglobulins: globulins with a molecular weight of over 1,000,000 appear in the blood. The disease mainly affects older people. In clinical practice, hemorrhagic syndrome predominates, sometimes with extremely heavy nosebleeds. The mechanism of hemorrhagic syndrome is complex and not fully established. It is believed that it is associated, on the one hand, with the inferiority of platelets interacting with macroglobulins, and on the other, with increased permeability of the vascular walls due to their infiltration by pathological proteins, high blood viscosity and intravascular agglutination of erythrocytes.

Mainly allocated skeletal forms and skeletal-visceral forms of the disease. In pathogenetic terms, the clinical picture of the disease comes down to two syndromes, namely bone damage and pathology of blood proteins. Bone damage is manifested by pain, fractures and the development of tumors. The spine, pelvic bones, ribs and skull are especially often affected, with the development of corresponding neurological symptoms.

Visceral pathology manifests itself mainly affecting the liver, spleen, lymph nodes and kidneys. Its development is associated both with specific cellular infiltration of these organs and with pronounced changes in blood proteins, with the accumulation of an abnormal protein in the blood - paraprotein produced by myeloma cells. In multiple myeloma, proteinemia can reach 12-18 g%.

Retinopathy in initial forms of myeloma and Waldenström's disease is absent. In a number of patients, the fundus presents a unique picture of fundus paraproteinemicus. Characterized by dilation of the retinal veins and an increase in their tortuosity. The arteries also dilate, but to a much lesser extent. Then the symptom of chiasm appears (compression of a vein under an artery), microaneurysms, occlusion of small veins, and hemorrhages in the retina. In some cases, cotton wool-like lesions in the retinal nerve fiber layer and papilledema also occur.

It is believed that these changes in the retina are associated with both hyperparaproteinemia and high blood viscosity. In the azotemic stage of the disease, retinopathy, characteristic of chronic kidney disease, develops.

As for changes in retinal vessels, their connection with increased blood plasma viscosity has been demonstrated experimentally. After injecting monkeys with dextran with a high relative mass, dilated and tortuous retinal vessels, especially veins, microaneurysms and hemorrhages, were detected in the fundus of the eye.

Multiple myeloma can affect also the orbital bones, eyelids, lacrimal gland, lacrimal sac and conjunctiva, infiltrate the sclera, iris, choroid, retina and optic nerve. These lesions, however, are not associated with increased blood viscosity.

Hemorrhagic diathesis

Hemorrhagic diathesis refers to such pathological conditions that manifest themselves increased bleeding in the absence of significant damage to the vascular wall, i.e. bleeding develops in situations where other healthy people in this regard do not have them.

Importance of the problem hemorrhagic diathesis is very high. Firstly, this is explained by the fact that the number of people suffering from increased bleeding in the world has exceeded six figures. Secondly, people suffering from hemorrhagic diathesis cannot be considered full-fledged members of society, since their potential capabilities are sharply limited both by anemia, which often accompanies this pathology, and by those types of activities that protect the patient’s blood vessels from various damages.

Thirdly, the importance of information about the presence of hemorrhagic diathesis in patients is determined by the fact that many forms of this suffering occur latently or manifest themselves weakly, having a monosymptomatic clinic. If surgical interventions are necessary, even minor ones such as tooth extraction or tonsillectomy, as well as when certain medications are prescribed, such as acetylsalicylic acid, hemorrhagic diathesis can threaten the patient’s very life.

The pathogenesis of hemorrhagic diathesis can currently be considered quite well studied. As is known, limit bleeding in a healthy person, when the vascular wall is damaged, it is carried out due to the following mechanisms: contraction of the vessel at the site of its damage, settling of circulating platelets at the site of damage to the vessel and the formation of a primary hemostatic plug by them and securing it with a fibrin wall with the formation of the final “secondary” hemostatic plug. Violation of any of these mechanisms leads to disruption of the hemostasis process and the development of hemorrhagic diathesis.

Modern ideas about the mechanisms of blood coagulation allow us to propose the following working classification of hemorrhagic diathesis.

CLASSIFICATION OF HEMORRHAGIC DIATHESES

I. Hemorrhagic diathesis caused by a defect in procoagulants (hemophilia):
a) insufficient amount of one or more factors involved in the formation of fibrin;
b) insufficient activity of procoagulant factors;
c) the presence of inhibitors of individual procoagulants in the patient’s blood.
II. Hemorrhagic diathesis caused by a defect in the platelet component of hemostasis:
a) insufficient number of platelets (thrombocytopenia);
b) functional inferiority of platelets (thrombocytopathy);
c) a combination of quantitative and qualitative platelet pathology.
III. Hemorrhagic diathesis, manifested as a result of excessive phpbrinolysis:
a) endogenous;
b) exogenous.
IV. Hemorrhagic diathesis, manifested as a result of pathology of the vascular wall:
a) congenital;
b) purchased.
V. Hemorrhagic diathesis, developing as a result of a combination of several causes (thrombohemorrhagic syndrome, von Willebrand disease).

The most common cause hemorrhagic diathesis is a defect in the platelet component of hemostasis, which is the cause of bleeding in 80% of patients [Marquardt F., 1976]. In the group of patients with hemorrhagic diathesis developing due to the inferiority of the procoagulant component of hemostasis, hemophilia A (65-80%), hemophilia B (13-18%) and hemophilia C (1.4-9%) are most often diagnosed.

Historically, hemorrhagic diathesis caused by fibrin formation defect. It is now known that fibrin formation is ensured by the correct interaction of procoagulant proteins, most of which have their own number, indicated by a Roman numeral. There are 13 substances, including fibrinogen (factor I), prothrombin (II), proaccelerin-accelerin (V), proconvertin (VII), antihemophilic globulin A (VIII), Christmas factor (IX), Stewart-Prower factor (X) , plasma thromboplastin precursor (XI), Hageman factor (XII), fibrin-stabilizing factor (XIII). Apart from them, three recently discovered factors do not have a numerical designation. These are the Fletcher, Fitzgeralz and Passova factors.

A quantitative or qualitative defect in any of the above procoagulants, as well as the appearance of an inhibitor of this factor in the patient’s blood, can cause a hemorrhagic condition in the patient.

The large number of these conditions, which approaches the number 30, as well as the great similarity of their clinical manifestations, make it possible to combine these diseases under the general name “ hemophilia».

Hemophilia is characterized by extensive, deep, usually isolated, spontaneous bruises and hematomas, frequent hemorrhages in the joints with the extremely rare development of skin and mucous “purpura” in rare and mild bleeding with superficial damage to the skin. Crude laboratory tests demonstrate prolongation of blood clotting time in the absence of bleeding time abnormalities. Practitioners must clearly understand that accurate diagnosis of the cause of hemorrhagic diathesis is possible only with the use of special laboratory research methods, without which adequate therapy is practically impossible.

Among the hemorrhagic diathesis that develops due to the inferiority of the platelet component of hemostasis, the most common are those that are caused by decreased platelet count in the patient's bloodstream. These conditions, called Werlhoff syndrome, are heterogeneous in their cause. The number of platelets can decrease both as a result of the formation of autoantibodies against them (autoimmune thrombocytopenia) and as a result of their inadequate formation in the bone marrow. Inferiority of the platelet membrane and their cytolysis are also possible.

In recent years, the attention of clinicians has been focused on such hemorrhagic conditions; which are caused by the functional inferiority of platelets, which are unable to provide complete hemostasis even with a sufficient number of them in the patient’s bloodstream. After this pathology was first described by Glyantsman, a large number of pathological forms were discovered that were caused by a violation of one or another stage of platelet plug formation carried out by platelets: their adhesion, aggregation, activation of the procoagulant link, retraction of a blood clot.

Detection of these defects, the identification of their combinations with some other manifestations of the disease led to the description of a number of individual nosological forms. At the same time, the study of platelet functions in a number of described diseases made it possible to note the absence of a connection between disorders of platelet functions and other symptoms not related to hemostasis.

Various combinations of defects in platelet functions made it possible to speak about the presence of a whole groups of thrombocytopathies, manifested by a wide variety of disorders of platelet functions such as adhesion, aggregation, release reaction, activation of procoagulants, retraction. When clarifying the cause of hemorrhagic diathesis, a detailed study of both the quantitative and qualitative state of platelets in the laboratory is necessary.

The clinical picture of these diseases is characterized by: frequent prolonged bleeding with superficial skin lesions, skin and mucous “purpuras” are common, while hemorrhages in the joints, spontaneous bruises and hematomas are quite rare.

Hemostasis defects caused by pathology of the vascular wall are diagnosed quite easily in cases where this pathology is accessible to visual observation: with Randu-Osler disease, Ehlers-Danlos syndrome, Hippel-Lindau disease, Kasabach-Merritt syndrome, etc. Currently, there are indications that hemorrhagic diathesis can develop when the collagen of the vascular wall is inadequate and platelet adhesion is impaired as a result. However, this pathology can only be diagnosed using complex laboratory methods.

Recently, clinicians have received a lot of attention cases of hemorrhage development in patients with multiple microthrombosis of the capillaries of internal organs. These conditions are called thrombohemorrhagic syndrome. Its pathogenesis is explained by the fact that with massive rapid thrombus formation in the clot, many blood coagulation factors are consumed, especially platelets and fibrinogen. In addition, hypoxia of the vascular wall leads to the release of a large number of plasminogen activators into the bloodstream and an increase in fibrinolytic activity of the blood. Diagnosis of these conditions is very important, as it requires the “paradoxical” use of anticoagulants for the treatment of hemorrhages.

Interesting finds were discovered during the study pathogenesis of bleeding in patients with von Willebrandt disease, which is characterized by a combination of symptoms reflecting disorders of both the procoagulant and platelet components of hemostasis. It was revealed that the factor VIII antigen is essential for triggering platelet adhesion to the damaged surface and showed the importance of the relationship between these leading mechanisms for stopping bleeding.

The wide variety of causes of hemorrhagic diathesis and the creation of specific methods for treating these conditions oblige practitioners to conduct a detailed study of the issues of diagnosis and treatment of patients with increased bleeding.

The most common eye manifestations of purpura are subcutaneous and conjunctival hemorrhages. Retinal hemorrhages are very rare. In cases where they do exist, hemorrhages are located in the layer of nerve fibers. It should be borne in mind that with eye trauma, including surgical trauma, heavy bleeding is possible, especially in hemophilia.

Blood diseases represent a fairly large and diverse group of diseases, accompanied by structural or functional disorders, pathological changes in the number of blood cells such as leukocytes (white blood cells that fight infections), platelets (due to which the blood tends to clot) and erythrocytes (red blood cells). oxygen-carrying bodies). Blood diseases also affect the liquid part of the blood - plasma.

Typical examples of blood diseases caused by changes in the number of cellular elements are, for example, anemia or erythremia (increased number of red blood cells in the blood). An example of a blood disease caused by changes in the structure and functions of cellular elements is sickle cell anemia, “lazy white blood cell” syndrome, etc. Pathologies in which the quantity, structure, and functions of cellular elements change are hemoblastoses, which are commonly called blood cancer.

Blood diseases

The pathogenesis of diseases of the blood and hematopoietic organs is studied by the science of hematology (haematologia). Depending on the etiology of occurrence and class, the main types of disorders are distinguished:

• red blood cell diseases;
• leukocyte pathologies;
• platelet diseases;
• incoagulability of blood.

Each group combines a large list of systemic and autoimmune diseases. In total, there are about 100 pathological abnormalities in the process of hematopoiesis.

Descriptions of blood diseases

Causes of blood diseases

The causes of blood diseases are different. Thus, diseases associated with the problem of blood clotting are usually hereditary. They are diagnosed in young children.

All infectious blood diseases, the list of which includes malaria and other diseases, are transmitted through the carrier of the infection. It could be an insect or another person.

The cause of blood disease can also be irradiation, radioactive or toxic poisoning. Anemia can occur due to poor nutrition, which does not provide the body with the necessary elements and vitamins.

Symptoms of blood diseases

The greatest danger in the course of blood diseases is the difficulty of early diagnosis, since most of the symptoms are not specific to this nosological group, and the patient most often attributes various types of ailments to overwork, seasonal vitamin deficiency and considers them to be a passing phenomenon.

A blood disease can be suspected by the following signs:

Diagnosis of blood diseases

In the diagnosis of blood diseases, many laboratory and instrumental research methods are used. Various blood tests are of particular importance. Further, during treatment, diagnostic procedures are carried out with greater frequency to monitor the dynamics of treatment and determine the effectiveness of therapy.

Treatment of blood diseases

Therapy for blood diseases is determined by diagnosis.

For anemia:

Leukemia is treated in a hospital using various cytostatic drug regimens (the choice is based on the type of disease).

Therapy for thrombocytopenia is based on the use of glucocorticoids and chemotherapy with alkaloid drugs. If indicated, splenectomy may be performed. For thrombocytopathies, hemostatic agents (dicinone, etamsylate) and vascular strengthening drugs (vitamin C, rutin) are prescribed. Patients are prescribed a special diet.

Coagulopathies are treated by replacing the deficient factor with a donor one. People with congenital coagulopathies should always carry a patient’s card with them so that if massive bleeding occurs, the ambulance and hospital staff know what can be done to stop it.

Prevention of blood diseases

Prevention of blood diseases consists of maintaining a healthy lifestyle and limiting the influence of negative environmental factors, namely:

Questions and answers on the topic "Blood diseases"

Question:Hello! I have a 1.3 month old child with hemoglobin level of 95, I took the iron supplement Hexavin. It doesn't rise any higher. What products would you recommend to us? The child is breastfed.

Answer: If your child’s anemia is associated with a lack of iron, then it is impossible to replenish it from any food products and eliminate anemia once it has already developed. In this case, treatment with iron supplements only is indicated. Diet therapy is an auxiliary method of treating anemia and becomes important to prevent its re-development only when the anemia is eliminated. The drug Hexavit is not an iron supplement, but rather a multivitamin without mineral complexes. You can choose the optimal iron supplement for your child, as well as the adequate dosage and duration of treatment, by scheduling a consultation with a pediatric hematologist.