Hemorrhagic diathesis propaedeutics. Hemorrhagic diathesis: classification, causes, symptoms and treatment, clinical recommendations. Hemorrhagic diathesis. Classification

Hemorrhagic syndrome

Bleeding

Laboratory data

Leukemia

Clinic acute leukemia

5 periods: pre-leukemic, acute, remission, relapse and terminal.

The skin is pale, characterized by hemorrhoids.S. (hemorrhages - from petechiae to ecchymosis), bleeding from the mucous membranes, typically the periphery of the lymph nodes, especially the cervical, submuscular, and inguinal. Rare manifestations of acute leukemia – change skin and subcutaneous cells in the form of leukemoids, necrotic. defeat skin and mucous membranes of the mouth. For acute leukemia may involve the lungs, central nervous system, and sex. organs and eyes, but this is typical for relapses of the disease, but if these symptoms appear. in the beginning. period - then this is a bad prognosticator. sign. Changes in the lungs occur under the “mask” of bronchitis, pneumonia, and pleurisy. X-ray revealed diffuse. changes in the interstitium of the lungs in the form of darkening.

Damage to the central nervous system (neuroleukemia) is characterized by leukemic “meningitis”, “meningoencephalitis”, or “encephalitis”. Common to all forms is the general brain. and mening. symp-we, clonic-tonic convulsions. Characteristic but striking. ChMN. Leukemia defeat in the hypothalamic region occurs in the form of diencephalic S. (thirst, polyuria, obesity, hyperthermia). Involvement of the genital organs in the leukemia is determined by palpation and compaction of the testicles and spermatic cords in boys and ovaries in girls.

Syndromes: intox, anemia, bone joint, proliferative (hyperplastic), hemorrhoids (myeloblastic).

In case of lymphoid variants, the proliferation of leukemia is obstructed. it takes place not only in the bone marrow, but also outside it, appearing in the periphery. l / u, liver and spleen.

For acute myel. leukemia proliferation of leukemia. as only in the bone marrow, > expressed S. bone marrow deficiency present. blastn. infiltration, anemia, granulocytopenia with inf. complication.

In children of the 1st year of life, signs of delay. leukemia can begin with t 0, sudden appetite (anorexia), increasing pallor, dyspnea. Ostr. leukemia in children under 1 year of age is severe with extramedullary localizations of the disease, regardless of the type of disease.

Variants of the course of exacerbation (relapse) acute. leukemia in children is distinguished by its diversity, these may be cases similar to /I/ the acute period, but there may be first changes only in the bone marrow puncture, without a clinic.

Diagnostics: The main thing is the sign of blast cells in the bone marrow punctate and their appearance in the peripheral blood. In the hemogram: NV, E-ov, platelets. Depending on the number of leukocytes, cases with the Nth number of leukocytes, a decrease (1.0 x 10 9 / l and<), повышенным (20,0 х 10 9 /л до 1,0 х 10 12 /л) кол-вом лейкоцитов.

The absolute indicator is the appearance of blast cells. However, there may be cases where they are absent in the periphery. blood.

Hemorrhagic diathesis. Classification, etiology, pathogenesis, clinic, diagnosis, treatment. VOLODICHEVA ELENA MIKHAILOVNA Chief hematologist of the Tula region Candidate of Medicine. sciences 1

Hemorrhagic diathesis syndrome n Hemorrhagic diathesis is a syndrome whose main clinical signs are increased bleeding, a tendency to repeated bleeding and hemorrhages, spontaneous or after minor injuries. 2

n Hemorrhages occur when the hemostatic system is disrupted. n Hemostasis is a biological system that ensures, on the one hand, the preservation of the liquid state of the blood, and on the other, the prevention and stopping of bleeding by maintaining the structural integrity of the walls of blood vessels and fairly rapid thrombosis of the latter. 4

Components of the hemostasis system: Plasma coagulation factors There are 13 blood coagulation factors in blood plasma n Cellular coagulation factors. Platelets participate in all phases of the hemostatic process. Erythrocyte and leukocyte coagulation factors were also identified n Vascular component. damage to the vascular wall leads to the release of active thromboplastic substance. . n 6

The first way is a violation of the platelet component of hemostasis - a decrease in the number of platelets (thrombocytopenia) or a violation of their functional state (thrombocytopathy). Most often in clinical practice, thrombocytopenic purpura (Werlhof's disease) occurs. The second way is a violation of the plasma pathogenesis due to a deficiency of clotting factors or the anticoagulant system - coagulopathy. A typical representative of this group is hemophilia. The third way is disorders of the vascular wall, vasopathy; hemorrhagic vasculitis. 7

Classification. 1. Coagulopathies: n Hemophilia A, B, C n Hypoconvertinemia, deficiency of factors V, III, X, XIII 2. Disorder of the megakaryocyte-platelet system n Thrombocytopenia (ITP, symptomatic) n Thrombocytopathies 3. Disorder of the vascular system: n Hemorrhagic vasculitis n Disease Randu-Osler. 8

Clinical types of bleeding n The clinical manifestations of hemorrhagic diathesis are based on hemorrhagic syndrome. A carefully collected anamnesis and objective examination allow us to identify the type of bleeding, which is of great importance for the differential diagnosis of hemorrhagic diathesis. There are five clinical types of bleeding. 9

n n n n The hematoma type is characterized by the following symptoms: - massive, deep, intense and painful hemorrhages in large joints, muscles, under aponeuroses and fascia, in subcutaneous and retroperitoneal tissue; - profuse spontaneous post-traumatic or postoperative bleeding, including from internal organs (gastrointestinal, renal), which often does not occur immediately after surgery or injury, but several hours later. The petechial-spotted (bruised) type is characterized by: - ​​superficial hemorrhages in the skin, they are not tense, painless, do not compress or destroy surrounding tissues; - bruises on the skin, which are larger in size than petechiae, but also not tense and painless; Petechiae and bruises occur spontaneously or with the slightest trauma. - gingival, nasal and uterine bleeding. 10

n n n n n The mixed, bruise-hematoma type is characterized by: - ​​petechial rashes and bruises that occur before hematomas; - hematomas in the retroperitoneal and subcutaneous tissue, as a rule, are not numerous, but large in size, there are practically no hemorrhages in the joints and their deformation. The vasculitic purpuric type is characterized by: - ​​hemorrhagic skin rashes, most often symmetrical; the elements of the rash are limited, slightly raised above the skin, their appearance is often preceded by blisters or vesicles, which are then soaked in blood; hemorrhagic elements can merge, the epidermis above them becomes necrotic with the formation of a crust; after the rash disappears, pockets of skin pigmentation remain; - bleeding from internal organs - gastrointestinal, renal. The angiomatous type is characterized by: - ​​persistent and repeated bleeding of one or two, rarely more, localizations (for example, nasal, pulmonary); - absence of spontaneous and post-traumatic hemorrhages in the skin and subcutaneous tissue. eleven

Internal pathway Contact surface External Tissue factor XII ICH VII XI XII IX insoluble fibrin Xa Va Phospholipids Ca² Prothrombinase XIII Soluble fibrin Prothrombin Thrombin Fibrinogen Fibrin monomer 13

Modeling of hemostasis pathways Intrinsic pathway: n Activated partial thromboplastin time (aPTT). External pathway: n Prothrombin time (1935, Quick). FV-mw 330,000, FVIImw 50,000, FX-mw 58,800 and FII Indicative tests: prothrombin time (PT); APTT; Thrombin time; Quantitative determination of fibrinogen 14

Prothrombin index - determined in the presence of calcium ions and excess tissue thromboplastin Clot formation depends only on the activity of the prothrombin complex (factors: II, V, VII and X) Prothrombin activity according to Quick 0.1 ml of plasma + 60 s Prothrombin index: donor / patient ratio · 100% 0.2 ml TCS* *- thrombin-calcium mixture 15

Interpretation of results Reason for prolongation of PT: n Administration of oral anticoagulants; n Liver diseases; n Vitamin K deficiency; n ICE; n Hereditary prothrombin deficiency, f. VII, X or V. Shortening of PT is observed with thrombosis 16

APTT – characterizes the internal pathway of hemostasis (1953): Factors VIII, IX, XII; n Prekallikriin (Fletcher factor) n High molecular weight kininogen (Fitzgerald factor) The test is sensitive to deficiency of all blood coagulation factors, except VII, to heparin, to specific and nonspecific inhibitors. n 0.1 plasma + 0.1 erylide/kaolin 3 min + 0.1 Calcium chloride 17

Interpretation of results Reason for prolongation of APTT: Deficiency of intrinsic coagulation pathway factors; Presence of clotting inhibitors; Liver diseases; Vitamin K deficiency; ICE; Administration of heparin. Shortening is observed in thrombosis and disseminated intravascular coagulation 18

Hemophilia A, B, C. Hemophilia is a congenital coagulopathy characterized by a deficiency of factors: n VIII (hemophilia A) n IX (hemophilia B, Christmas disease) n XI (hemophilia C). 19

Hemophilia is a disease characterized by a quantitative or qualitative deficiency of coagulation factors VIII (hemophilia A), IX (hemophilia B) n n n Antihemophilic factor VIII (Factor VIII) MB 270,000 - 340,000 is synthesized by endothelial cells of the liver? VIII: C is associated in plasma with von Willebrand factor FVIII - internal pathway normal concentration about 0.5 μg/ml n n n Factor IX (Christmas factor) MV 72,000 synthesized in the liver thermally labile and shelf-stable vitamin K-dependent normal concentration 3 μg /ml 20

The history of hemophilia goes back to the distant past. The first mention of hemophilia is contained in the Babylonian Talmud (1500 years ago). The term hemophilia appeared in 1823 21

Etiopathogenesis. n Hemophilia A is 80% n Hemophilia B is 19% n Hemophilia C is 1% Hemophilia A and B are transmitted recessively, linked to the X chromosome. Mostly men are affected, women are carriers of the gene and half of their sons may suffer from hemophilia. Hemophilia C is transmitted in an autosomal recessive manner, so individuals of both sexes are affected with equal frequency. 22

wives N R F 1 h X X N N X X, N h XX, healthy. wears. husband. --H X Y, healthy. n X Y h XY patient 23

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Population of Russia and the estimated number of patients with hemophilia 6,764 67,645,000 77,654,000 According to the State Statistics Committee, the results of the All-Russian Population Census of 2002 The country has a population of 145 million 290 thousand people 26

The severity of bleeding depends on the degree of factor deficiency. Normally, it is 50 -100% Latent form n When reduced to 20 -50%, there is a tendency to increased bleeding in large injuries Expressed form n At a factor level of 5 -20%, severe bleeding occurs in injuries Severe form n If it fluctuates within from 1-5%, spontaneous bleeding occurs. Very severe form n Complete absence of the factor. n 27

Clinic. Increased bleeding appears from the first months of a child’s life n Bleeding appears against the background of injuries: cuts, bruises, various impingements n Deep hemorrhages occur, heavy bleeding at the site of injury, hemorrhages in large joints (hemarthrosis), leading to the development of contracture and ankylosis of joints (often knee and ankle), dangerous massive intermuscular, subfascial, retroperitoneal hematomas, humaturia. n 28

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Diagnostics. 1. n n 2. 3. Coagulogram: Decreased blood clotting time Increased active partial thromboplastin time Decreased autocoagulation, coagulation activity Prothrombin and thrombin times are normal. Decrease in the level and activity of factors VIII, IX, XI. Immunological test to determine the factor antigen using homologous inhibitor antibodies. 35

What is factor VIII activity? Measured in international units per vial (250 IU, 500 IU, 1000 IU); n In 1 ml. normal plasma contains 1 IU of factor VIII; n 1 IU factor VIII administered per 1 kg. body weight increases factor VIII levels by ~2%. n 38

Therapeutic dose: Hemophilia A – 25 -50 IU/kg every 12 hours; n Hemophilia B – 25 -50 IU/kg every 24 hours; n 39

Replacement therapy for blood coagulation factors VIII or IX is carried out by the patient himself or after hemorrhage or for prophylactic purposes. 40

Treatment. FFP containing factors VIII, IX, XI 2. Cryoprecipitate (about 100 units in 1 serving) 3. Antihemophilic plasma (concentrate of factors VIII, IX), agemfil A, B Local therapy: 1. Minimizing pain, suturing, ice 2. For hemarthrosis - immobilization, ice, elevated position General therapy: 1. Epsilon - aminocaproic acid, synthetic progestins Treatment of anemia: erythritis transfusions. masses. 1.41

Etiopathogenesis of ITP. The main cause of bleeding is thrombocytopenia. Causes of thrombocytopenia: 1. Inhibitory effect of the spleen on platelet formation - inactive megakaryocytes are found in bone marrow aspirate. n 43

2. Accelerated destruction of platelets in the spleen - life expectancy of platelets is several hours instead of 7-10 days 3. In hereditary thrombocytopenia, defects in the membrane structure, disruption of energy processes in them lead to rapid destruction 4. With acquired ITP, antiplatelet antibodies are produced in the spleen. Ig. G. 44

Course of the disease: n Chronic, recurrent n Acute (hapten) Viral infection or certain medications (sulfonamides, butadione, quinine) play the role of a hapten associated with a platelet. The resulting antibodies cause the destruction of platelets and the occurrence of increased bleeding. 45

ITP Clinic. n The clinical picture appears when the platelet level is below 100 x10*9/l. If the platelet level is below 50 x10*9/L, life-threatening bleeding may occur. . 46

Pinpoint subcutaneous hemorrhages - petechiae, ecchymoses, located asymmetrically n Bleeding from the mucous membranes: gingival, nosebleeds n Bleeding from the gastrointestinal tract n Hematuria n Hemoptysis n Hemorrhages at injection sites n Prolonged bleeding after tooth extraction n Posthemorrhagic anemia. n 47

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Diagnosis of ITP. n n n General blood test: Decreased number of red blood cells and hemoglobin Decreased number of platelets (below 100 x10*9/l) In an autoimmune process, increased ESR Morphological changes in platelets: increase in size. 50

n Coagulogram: increased bleeding according to Duke and Ivey, decreased retraction, blood clotting time and kaolinkephalin test are normal n Myelogram: the number of megakaryocytes is increased n Dixon test - detection of antiplatelet antibodies. 51

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Treatment of ITP. Glucocorticosteroids: prednisolone 40-60 mg/day according to the regimen n If GCS is ineffective, immunosuppressants are used - metipred, vincristine, azathioprine, cyclophosphamide n Splenectomy Indications: increasing anemia, ineffectiveness of drugs. . n 53

n Thrombocytopathies are diseases in which the qualitative and functional properties are impaired - adhesion and aggregation of platelets, leading to impaired hemostasis. In thrombocytopathies, the platelet count is within normal limits. n Among hemorrhagic diathesis, it occurs in 36% of cases. 54

Clinic of thrombocytopathy. Subcutaneous petechial-spotted hemorrhages, hematomas located asymmetrically n Bleeding from the mucous membranes: gingival, nasal bleeding n Bleeding from the gastrointestinal tract n Hematuria n Hemoptysis n Hemorrhages at injection sites n Prolonged bleeding after tooth extraction n Posthemorrhagic anemia. n 55

Diagnostics. n General blood test: 1. Decrease in the number of erythrocytes and hemoglobin 2. The number of platelets is normal 3. Granulomeres and processes are not determined in the morphology of platelets. 56

Treatment. Diet with vitamin C, P, A, consumption of peanuts n Aminocaproic acid 6 -12 g/day n Dicynone, vikasol n PAMBA, tranexam acid n ATP, magnesium sulfate n Riboxin, inosine-F n Androxon, adrenoxyl n Platelet mass n Local stop blood. n 61

Pathogenesis. Hemorrhagic vasculitis belongs to the immune complex diseases, as it is caused by the damaging effect of low molecular weight immune complexes (IC). When they are formed, a predominance of the antigen is observed. IR and the complement activated by them cause microthrombovasculitis with fibrinoid necrosis, perivascular edema, and blockade of microcirculation. 64

Clinical course. According to the clinical course, they are distinguished: n Cutaneous or simple form n Articular form n Abdominal form n Renal form n Fulminant form According to the course of the disease, they are distinguished: 1. Acute 2. Chronic, recurrent. 65

Clinic. n Cutaneous form Skin lesions: small punctate, symmetrical petechiae, mainly on the lower extremities and buttocks. The rash is monomorphic, with a distinct inflammatory basis, lasts 4-5 days, leaving behind pigmentation. 66

n Articular form n The site of joint damage is the synovial membrane. n There is severe pain, swelling, dysfunction of the joint n Abdominal form Hemorrhages occur in the mucous membrane of the stomach, intestines, and mesentery. Severe abdominal pain occurs, simulating the picture of an acute abdomen, body temperature may rise, and sometimes vomiting occurs. Blood is detected in the stool. 67

n Renal form n Proceeds as acute or chronic nephritis, sometimes takes a protracted form and turns into chronic renal failure. Possible development of arterial hypertension, nephrotic syndrome n Fulminant (cerebral) form Develops with hemorrhage in the membranes of the brain or vital areas. 68

Diagnostics. n Clinical manifestations: monomorphic small-pointed, symmetrical rashes that do not disappear with pressure n Hyperfibrinogenemia, increased content of IR, cryoglobulins, gammaglobulins n Positive autocoagulation test n On the coagulogram, a shortening of the total blood clotting time, prothrombin and thrombin time. 69

Treatment: Heparin 7500 -15000 IU/day IV or SC under the control of blood coagulation n Heparinoids: sulodexide, loloparan n Staged plasmapheresis n Steroid hormones: prednisolone 20 -60 mg/day according to the scheme n Improvement of microcirculation: trental, pentoxifylline n Strengthening the vascular wall: ascorbic acid, rutin. n 70

Hemorrhagic diathesis– pathological conditions characterized by increased bleeding as a result of insufficiency of one or more elements of hemostasis.

Etiopathogenetic classification of hemorrhagic diathesis:

a) hereditary– are associated with genetically determined pathological changes in the vascular wall, abnormalities of megakaryocytes, platelets, adhesive plasma proteins and plasma factors of the blood coagulation system.

b) purchased– due to the following reasons:

1) due to primary damage to the vascular wall: hereditary hemorrhagic telangiectasia of Randu-Osler; Henoch-Schönlein hemorrhagic vasculitis; Ehlers-Danlos syndrome, hypovitaminosis C and B, etc.

2) due to primary damage to the megakaryocyte-platelet lineage:

1. thrombocytopenia(idiopathic thrombocytopenic purpura, increased consumption of platelets in DIC, redistribution of platelets and their deposition in the spleen)

2. thrombocytopathies(Glyantsman thrombasthenia, von Willebrand disease)

3) due to blood clotting disorders (coagulopathy): vitamin K-dependent (with insufficiency of liver function, impaired absorption of vitamin K, nutritional deficiency of vitamin K, etc.); liver failure with deficiency of coagulation factors, etc.; pathological clotting inhibitors (“lupus anticoagulant”)

4) due to complex disorders of various parts of the coagulation system: acute disseminated intravascular coagulation syndromes

Types of bleeding:

1) capillary (microcirculatory, petechial-spotted, bruised)– petechial rashes, bruises and ecchymoses on the skin and mucous membranes; often combined with bleeding of mucous membranes - nosebleeds, menorrhagia (thrombocytopenia, thrombocytopathy)

2) hematomatic– painful, intense hemorrhages in the subcutaneous tissue, muscles, large joints, peritoneum and retroperitoneal space; sometimes renal and gastrointestinal bleeding (hemophilia A and B)

3) mixed capillary-hematoma (bruise-hematoma)– petechial bruises in combination with extensive dense hemorrhages and hematomas; hemorrhages in the joints are not typical

4) vasculitic purpuric– hemorrhagic or erythematous (inflammatory based) rashes of varying sizes; easily occur in places where the skin is compressed by a belt or socks (vasculitis)

5) angiomatous– persistent, strictly localized and associated with local vascular pathology bleeding (telangiectasia, hematomas)

Thrombocytopenic purpura– a group of diseases united according to the principle of a single pathogenesis of thrombocytopenia (shortened life expectancy of platelets due to the action of antibodies to them or other mechanisms of their destruction).

Normally, the platelet count is 150-450/μl, their minimum level is critical franc figure– 30/µl (below this, spontaneous hemorrhages can be expected).

Etiology of idiopathic thrombocytopenic purpura unknown, at the basis of pathogenesis– fixation on the surface of platelets of IgG directed against the antigens of one’s own platelets ® increased phagocytosis of platelets by macrophages of the spleen and liver ® increased destruction of platelets, shortening their life expectancy (from 7-10 days normally to several hours)

There are acute (usually in children 2-6 years old, lasts no more than 6 months, characterized by a rapid, sudden onset, severe hemorrhagic syndrome followed by spontaneous recovery or remission) and chronic (in adults, lasts several years) forms of the disease.

Clinic of the chronic form of idiopathic thrombocytopenic purpura.

The disease is most typical for women, develops gradually, gradually, is chronic, relapsing in nature with alternating periods of exacerbation and periods of remission of varying durations

Hemorrhagic syndrome:

a) bleeding of the petechial-spotted type in the form of skin hemorrhages, most often localized on the anterior surface of the body, upper and lower extremities, and at injection sites; the color of hemorrhagic rashes changes depending on how long ago they appeared: first purplish-red, then bluish, greenish, yellow (“blooming bruises”)

b) bleeding from the mucous membranes: nasal, gingival, polymenorrhea, in severe cases - renal (macrohematuria), pulmonary (hemoptysis), gastrointestinal (melena, vomiting “coffee grounds”) and other bleeding

Intracerebral and subarachnoid hemorrhages, hemorrhages in the sclera or retina, severe bleeding after tonsillectomy, tooth extraction, during surgery and childbirth may be observed.

With frequent and heavy bleeding - signs of posthemorrhagic anemia (pallor of the skin and visible mucous membranes, etc.)

Diagnosis of thrombocytopenic purpura:

1. UAC: decrease in total platelet count< 100*10 9 /л, их морфологические изменения (анизоцитоз, пойкилоцитоз и шизоцитоз); преобладают тромбоциты больших размеров (3-4 нм в диаметре),

there are small platelets and platelet fragments (“microparticles”); hypochromic anemia; moderate neutrophilic leukocytosis with a shift to the left after heavy blood loss

2. Study of hemostasis: increase in bleeding time (up to 15 minutes or more when the norm is 2.0-7.5 minutes); blood clot retraction disorder

3. Immunogram: increased content of IgG to platelet antigens, increased circulating immune complexes

Treatment of thrombocytopenic purpura:

1. Prednisolone or methylprednisolone at an initial dose of 1 mg/kg/day ® no effect for 5-7 days ®

increasing the dose to 2-3 mg/kg/day (pulse therapy with methylprednisolone is possible); the duration of hormonal therapy is from 1-4 months to six months, hemorrhages stop in the first days of treatment, and platelets increase gradually

2. If GCS is ineffective within six months - splenectomy

3. If splenectomy is ineffective - chemotherapy (vincristine, azathioprine, cyclophosphamide in combination with prednisone)

4. It is possible to use large doses of human Ig intravenously (sandoglobulin 0.25 g/kg, then a maintenance dose of 0.5 mg/kg every 15 days) - immunoglobulin closes macrophage receptors and they stop absorbing platelets

5. Plasmapheresis to remove AT

6. Courses of treatment with dicinone (etamsylate) 1.5 g/day orally for 14 days

7. Platelet infusions are not indicated and are used only for health reasons

Hemorrhagic vasculitis (Scheilein-Henoch disease)– vasculitis, characterized by deposition in the walls of small vessels (arterioles, capillaries, venules) of IgA-containing immune complexes with characteristic symmetrical hemorrhagic rashes, arthritis, abdominal syndrome and glomerulonephritis.

Epidemiology: 1st place among systemic vasculitis; Children and young people under 20 years of age are more likely to get sick

Etiology of hemorrhagic vasculitis:

a) drug allergy

b) the use of serums and vaccines

c) insect bites

d) cold allergy

e) food idiosyncrasy (milk, eggs, strawberries, etc.)

Infectious agents (usually group A b-hemolytic streptococcus, mycoplasmas, viruses) are only a resolving factor, not a causative one.

Pathogenesis of hemorrhagic vasculitis: immune complex inflammation with the formation of circulating immune complexes (CIC) with IgA ® deposition of CIC in the microvessels of the skin and internal organs ®

destructive and destructive-productive microvasculitis with multiple microthrombosis, increased permeability of the vascular wall with the release of proteins and red blood cells from the vascular bed

Clinical picture of hemorrhagic vasculitis:

a) the onset is often acute, sudden, with an increase in temperature to subfebrile, weakness, malaise

b) skin syndrome– leading clinical syndrome present in all patients:

Small-spotted (2-3 mm in diameter), symmetrical hemorrhagic rash prone to fusion, easily identified visually and palpably

The rash is most often located on the extensor surface of the upper and lower extremities, on the buttocks, less often on the torso and almost never on the mucous membranes, worsens in an upright position

Elements of the rash disappear 2-3 days after appearance

Usually there are 2-4 waves of rashes, so both old and fresh elements are present on the skin at the same time (a mottled picture)

Confluent purpura can lead to the formation of hemorrhagic blisters, which then rupture to form deep erosions and ulcers

c) articular syndrome– occurs in 2/3 of patients, more often in adults:

Symmetrical damage to large joints, mainly the lower extremities (knee, ankle) with periarticular edema, pain, limited function, but without bone changes

A combination of arthritis with myalgia and swelling of the lower extremities is typical.

Duration of articular syndrome is 1-2 weeks

d) abdominal syndrome– in more than 50% of patients, it occurs due to edema and hemorrhages in the peritoneum, intestinal wall (the initial and final parts of the small intestine are more often affected, hemorrhagic and ulcerative-necrotic changes in the large intestine, damage to the esophagus and stomach are less often observed):

Sudden onset of intense abdominal pain similar to intestinal colic, localized in the mesogastrium, cramping, sometimes accompanied by nausea, vomiting (including bloody)

Typical gastrointestinal bleeding with tarry stools may develop

Complications: intussusception (more often in children), intestinal obstruction, perforation with peritonitis

Duration of abdominal syndrome from days to 10

e) renal syndrome– in 10-50% of patients, more often in adults:

Glomerulonephritis usually occurs in the first 4-6 weeks after the onset of the disease

The leading manifestations are isolated gross hematuria or its combination with moderate proteinuria; nephrotic syndrome and hypertension are uncharacteristic

With persistent hematuria and proteinuria, chronic renal failure may develop

e) pulmonary syndrome- capillaritis of the interalveolar septa with hemorrhages in the alveoli:

Cough with scanty sputum, hemoptysis, shortness of breath

Inconsistency between the scanty auscultatory picture and the degree of radiological changes (multiple infiltrates in the middle and lower sections)

Sometimes - hemorrhagic pleurisy

g) heart damage- hemorrhagic pericarditis, hemorrhages in the endocardium, infarction changes are possible on the ECG

h) damage to the central nervous system- paroxysmal headaches, dizziness, tearfulness, irritability, with swelling of the membranes - meningeal symptoms, epileptiform seizures, etc.

Clinical variants of hemorrhagic vasculitis:

a) fulminant form - death a few days later from a stroke or intestinal bleeding

b) acute form – from several weeks to several months; the outcome is recovery or relapsing course

c) relapsing course - characterized by relapses with periods of remission of varying duration (from several months to a year or more)

Diagnosis of hemorrhagic vasculitis:

1. Laboratory findings are non-specific:

a) OAC: moderate leukocytosis with a shift to the left, increased ESR (in the abdominal form and especially in GN); often eosinophilia up to 10-15%; platelets are normal

b) OAM: hematuria, proteinuria (with GN)

c) LHC: dysproteinemia in the acute period due to increased IgA

d) positive stool test for occult blood in abdominal syndrome

2. Instrumental studies:

a) skin biopsy and its immunohistochemical study - perivascular leukocyte infiltrates, deposition of IgA-containing immune complexes

b) FGDS - detection of erosions in the esophagus, stomach, duodenum, etc.

Treatment:

1. Bed rest, limiting intake of extractive, salty, spicy foods

2. The main method of treatment is heparin therapy: 300 units/kg/day subcutaneously (the dose is evenly distributed over several injections every 4-6 hours); control - thrombin time (optimal) or coagulation time (less sensitive indicator), it is necessary to achieve their extension by 2 times

3. If the effect of heparin is insufficient:

a) to replenish antithrombin III - FFP 300-400 ml IV

b) nicotinic acid 0.1% - 1 ml (1 amp) in saline. solution intravenously by slow drip to stimulate fibrinolysis

c) disaggregants – pentoxifylline/trental 2% solution 5 ml per 200 ml saline. solution intravenously drip

d) treatment of inflammation - NSAIDs, short courses of corticosteroids, for rapidly progressing GN - pulse therapy with methylprednisolone 1000 mg/day IV for 3 days

e) with a high level of CEC, long-term persistent course of vasculitis - plasmapheresis, immunosuppressants

Hereditary coagulopathies- genetically determined disorders in the blood coagulation system associated with deficiency or molecular abnormalities of plasma coagulation factors and components of the kallikrein-kinin system involved in this process; 97% of all hereditary coagulopathies are hemophilia.

Structure of hemophilia:

a) hemophilia A (85-90%)– coagulopathy, which is based on a deficiency of the coagulation part of plasma coagulation factor VIII - VIII:C (antihemophilic globulin A) or its molecular changes

b) hemophilia B / Christmas disease (6-13%)– coagulopathy, which is based on a deficiency in the activity of factor IX (plasma component of thromboplastin)

c) hemophilia C / Rosenthal disease (0.3-0.5%)– coagulopathy, which is based on a deficiency of coagulation factor XI (participates in the internal pathway of blood coagulation activation)

Etiology of hemophilia: hereditary diseases (hemophilia A and B are inherited in an X-recessive manner - predominantly men are affected, hemophilia C is inherited autosomal - both men and women are affected).

Pathogenesis: deficiency of blood coagulation factors leads to an increase in the clotting time of whole blood and the development of hemorrhagic syndrome (hematoma type of bleeding).

The severity of hemophilia is determined by the activity of the clotting factor in the blood:

a) mild form – activity more than 5%

b) moderate form – activity 3-5%

c) severe form – activity 1-2%

d) extremely severe form – activity less than 1%

Clinical manifestations of hemophilia:

The disease usually begins in childhood, affecting mostly boys (except for hemophilia C); mild hemophilia may begin in adolescence

The first symptoms are bleeding from minor injuries to the mucous membranes

Characterized by alternating periods of increased bleeding and relative well-being

Hematoma type bleeding:

a) heavy and prolonged bleeding after any, even minor, injuries and operations (tooth extraction, biting the lip and tongue, etc.)

b) hemarthrosis of large joints of the limbs with minor injuries (deterioration in general health, increased temperature, severe pain, hyperemic, tense, hot skin); recurrence of acute hemarthrosis leads to chronic hemorrhagic-destructive osteoarthrosis, ending in deformation and limitation of mobility, muscle wasting

c) large intermuscular, intramuscular, subperiosteal, retroperitoneal hematomas, causing destruction of surrounding tissues (hemophilic pseudotumors), leading to impaired mobility in the joints when the hematoma compresses nerve trunks, tendons, muscles

d) retrobulbar hematomas due to eye injury with loss of vision

e) hemorrhages in the brain and spinal cord

e) persistent renal bleeding (in 30% of cases)

g) gastrointestinal bleeding and tendency to ulceration

h) delayed (i.e. occurring after 1-5 hours) bleeding after injuries and operations

Anemic syndrome (weakness, pallor, dizziness, etc.) with prolonged bleeding

Diagnosis of hemophilia:

1. OAC: for extensive hematomas and bleeding – posthemorrhagic anemia of varying severity; normal platelet count

2. Coagulogram: duration of bleeding is normal (2.0-7.5 minutes); retraction of the blood clot is not impaired;

the total blood clotting time in a test tube is prolonged (normally 5-7 minutes), APTT is prolonged (the most important indicator for hemophilia, associated with factors VIII, IX and XI, normally 35-40 seconds), prothrombin time (characterizes the coagulation process when it is started by an external mechanism) - normal (11-14 sec) and thrombin time (characterizes the state of the final stage of the coagulation process) - normal (14-16 sec).

3. LHC: decreased activity of blood clotting factors (VIII, IX or XI depending on the type of hemophilia)

Treatment for hemophilia:

1. The primary task is hemotherapy replacement therapy, however, it is accompanied by a number of complications:

a) transmission of infection (HIV, hepatitis B, C)

b) pyrogenic and allergic reactions

c) formation of an inhibitor to the necessary coagulation factor (!)

d) transfer of non-viral pathogens (prions - transmissible spongiform encephalopathies: Creutzfeldt-Jakob disease and its variants, bovine spongiform encephalitis).

e) volume overload (cryoprecipitate and FFP only)

f) hemolysis, etc.

There are 3 generations of replacement therapy drugs:

I generation - cryoprecipitate and fresh frozen plasma (FFP)

II generation – concentrates of plasmatic coagulation factors of low and medium purity

III generation – concentrates of plasmatic coagulation factors of high purity, recombinant factors (recombinant and monoclonal purified factor VIII; monoclonal purified factor IX).

Required doses of factors for bleeding in hemophiliacs:

All antihemophilic drugs are administered intravenously immediately after they are re-opened! The half-life of factor VIII is 12 hours; for hemophilia A it is administered 2 times a day; The half-life of factor IX is 24 hours; for hemophilia B, it is administered once a day. Antihemophilic drugs cannot be diluted or administered intravenously with other blood substitutes (as their concentration decreases due to dilution)!!!

2. Due to replacement therapy possible formation of factor VIII inhibitor– neutralizing IgG (approximately 15% of patients with severe hemophilia A and 4% with hemophilia B); the inhibitor level is determined by the level of factor VIII remaining in normal plasma after 2 hours of incubation with the patient’s plasma, and is measured in Bethesda units (BU):

Low inhibitor titer (10 BU or less) – an increase in the amount of administered factor is indicated

High inhibitor titer (40 BU and above) – plasmapheresis is indicated (to remove IgG) + large dose of injected factor + GCS (prednisolone up to 4-6 mg/kg/day)

3. Other drugs for the treatment of hemophilia:

a) desmopressin intravenously for adults 1–4 mcg/day, children - 0.4 mcg/day - increases the concentration of factor VIII by 300-400% 5 hours after administration

b) prothrombin complex preparation (PPSP, autoplex, feiba), activated prothrombin complex (APPC) - contain several plasma coagulation factors (II, IX, X).

4. Stop bleeding: aminocaproic acid 4-12 g/day in 6 doses or other fibrinolysis inhibitors (pamba - aminomethylbenzoic acid, transamcha - tranexamic acid) + local hemostatic therapy (applications to the bleeding surface of a hemostatic sponge, lotions with a solution of thrombin, aminocaproic acid, etc. )

5. Treatment of hemarthrosis:

a) replacement therapy in the acute period (start as soon as possible)

b) bed rest 5-7 days

c) for severe hemorrhages: puncture of the joint with aspiration of blood and injection of corticosteroids into its cavity

d) immobilization of the affected limb for 3-4 days, then exercise therapy and physiotherapy (under the guise of replacement therapy)

clinical session

"HEMORRHAGIC DIATHESES"

Lesson duration: 4 hours Type of lesson - practical

Purpose and objectives of the lesson: to study the main clinical forms of hemorrhagic

diathesis in children, learn to recognize disorders in the hemostatic system, become familiar with modern principles of therapy and prevention of hemorrhagic diathesis. The student must know:

1. Etiology and pathogenesis of hemorrhagic diathesis in children

2. Classification of hemorrhagic diathesis

3. Leading clinical forms, symptoms, laboratory diagnostics

4. Principles of treatment

5. Prevention

6. Forecast

The student must be able to:

1. Identify complaints, collect and analyze the medical history and life of the patient

2. Examine the patient

3. Identify the leading clinical symptoms and syndromes

4. Draw up an examination plan

5. Evaluate the results of laboratory tests

6. Formulate a clinical diagnosis according to the classification
Outline a treatment plan

Main questions of the topic:

1. Physiological basis of hemostasis

2. Basics of diagnosing hemorrhagic diathesis

3. Classifications of hemorrhagic diathesis

4. Etiopathogenesis, clinical symptoms, principles of pathogenetic therapy, prevention and prognosis of the main forms of hemorrhagic diathesis:

Hemorrhagic diathesis caused by pathology of the vascular wall - immune microthrombovasculitis (Henoch-Schönlein disease)

Hemorrhagic diathesis caused by pathology of the platelet component of hemostasis - hemorrhagic thrombocytopenic disease (Werlhof's disease)

Hemorrhagic diathesis caused by a deficiency of plasma coagulation factors (hereditary coagulopathies) - hemophilia A, B (Christmas disease), C (Rosenthal disease), von Willebrand disease.

Self-study questions:

1. Structural inferiority of the vascular wall:

congenital hemorrhagic telangiectasia (Rendu-Osler disease) Louis-Bar sign

2. Congenital connective tissue diseases:

Marfan's sign

osteogenesis imperfecta (Lobstein's disease)

3. Acquired connective tissue lesions:

scurvy - steroid-induced purpura

4. Psychogenic purpura (Munghausen's symptom)

5. Vascular damage in various diseases: diabetes mellitus, varicose veins, diffuse agiokeratoma (Andresep-Fabry disease)

6. Neonatal alloimmune thrombocytopenic purpura (NATP)

7. Autoimmune thrombocytopsia (AIT11)

8. DIC syndrome

METHODOLOGICAL INSTRUCTIONS

Hemorrhagic diathesis is the general name for conditions characterized by increased bleeding.

Blood coagulation diagram

The schematic process of blood clotting can be divided into three phases:

1. prothrombin formation or contact-kallikrein - kenin - cascade activation. This phase leads to the formation of a complex of factors that can convert prothrombin into thrombin; this complex (factor Xa + factor Va + Ca ++ ions + platelet phospholipid) is called prothrombinase. There are two ways to activate this phase - external and internal. The first phase is the phase of prothrombinase formation, lasting from 4 minutes. 50 sec. up to 6 min. 50 sec.

2. the second phase, or the general path of thrombin formation - thrombin formation - the conversion of prothrombin into thrombin under the influence of prothrombinase, it lasts 2-5 seconds.

3. the third phase is fibrin formation, it lasts 2-5 seconds.

Along with the coagulation system, which ensures the formation of a blood clot, there is a system whose functioning is aimed at eliminating (lyzing) the blood clot. Fibrinolysis is important in wound healing and is also the body's way of combating blood vessel occlusion.

Fibrinolysis is a physiological process that eliminates insoluble fibrin deposits (fibrin clot) by enzymatic breakdown of stable fibrin polymers. Under the influence of plasmin, the clot dissolves.

There are plasma and cellular fibrinolytic systems.

Plasma fibrinolytic system The plasma fibrinolytic system includes: plasminogen (proenzyme)

plasminogen activators

plasmin (enzyme)

plasmin inhibitors

plasminogen activator inhibitors

Cellular fibrinolytic system

Leukocytes and macrophages are able to directly participate in fibrin lysis by releasing proteolytic enzymes. In addition, leukocytes and macrophages phagocytize fibrin and various cell fragments accumulated at the site of injury.

Blood clotting inhibitors - anticoagulant system

Along with the blood coagulation system, there is an anticoagulant system, represented by various blood clotting inhibitors. The blood coagulation system and the anticoagulant system are normally in a well-balanced relationship. The tasks of the anticoagulant system are to prevent the activation of coagulation factors, prevent the occurrence of massive intravascular thrombosis, and limit the coagulation reaction to the site of damage.

All anticoagulant substances formed in the body can be divided into two groups:

Primary anticoagulants are substances that are constantly synthesized, regardless of blood coagulation and fibrinolysis, and released into the bloodstream at a constant rate (antithrombin III, heparin, heparin cofactor II, αI-Antitrypsin, nexin-I protease, thrombomodulin);

Secondary anticoagulants are substances formed from blood coagulation factors and other proteins as a result of hemocoagulation and fibrinolysis (antithrombin I, metafactor Va, metafactor X1a, fibrinolysis products).

DIAGNOSIS OF HEMOSTASIS DISORDERS

Study of vascular-platelet hemostasis

Vascular component

Pinch test. The doctor collects the skin under the collarbone and makes a pinch. Normally, there are no changes either immediately after the pinch or during the day. As resistance decreases, petechiae or bruising appear, especially after 24 hours.

Tourniquet test or cuff test. A tonometer cuff is placed on the shoulder, maintaining the pressure at 90-100 mm. rt. Art. within 5 minutes. Then the cuff is removed and after 5 minutes the number of petechiae is counted on the inner surface of the forearm 2 cm downward from the elbow in a circle with a diameter of 5 cm. Normally, the number of petechiae does not exceed 10; 11-20 - weakly positive test; 20-30 - positive test; 30 or more is a sharply positive test. f

Platelet component

Determination of the number of platelets in the blood. The number of platelets in capillary blood is normally 150 - 350 x 10 /l.

Determination of the duration of bleeding (according to Duque). The duration of bleeding reflects the elasticity of blood vessels, their ability to spasm during injury, as well as the ability of platelets to adhesion and aggregation. The principle of the method is to determine the duration of bleeding from the microvessels of the skin (earlobe area after puncture with a lancet to a depth of 3.5 mm). Norm - 2 - 3 minutes. Prolongation of bleeding duration - with thrombocytopenia, thrombocytopathy, disorders (damage) of the vascular wall.

Determination of platelet aggregation function. Studied using an aggregometer. Normally (according to Weiss) - at a concentration of adenosine diphosphate (ADP) 10 µm/ml - 77.7%, at a concentration of 1 µm/ml - 30.7%. Aggregation decreases in congenital and acquired thrombocytopathies, throbocytopenia, hypothyroidism, and treatment with non-steroidal anti-inflammatory drugs. An increase is typical for systemic vasculitis and systemic connective tissue diseases.

Study of plasma (coagulation) hemostasis

Assessment of the first phase of blood coagulation -

phases of prothrombinase formation

Clotting time(according to Lee-White). The method consists of determining the rate of clot formation in venous blood at a temperature of 37°. The norm is 8-12 minutes, according to the micromethod - 5-10 minutes. A pronounced prolongation of blood clotting time is observed with a profound deficiency of blood coagulation factors, with thrombocytopenia, thrombocytopathy, and with heparin treatment. A shortened time indicates hypercoagulability.

Aactivated partial thromboplasty time (A PTT)

IN normal - 30-42 sec

Prolonged aPTT indicates hypocoagulation and is observed with a deficiency of all plasma factors except VII, and treatment with heparin and anticoagulants

Factor activity: normal

The autocoagulation test reflects the state of procoagulant and anticoagulant processes

Plasma recalcification time Normal 80-140 sec More than 140 sec - hypocoagulation Less than 80 sec - hypercoagulation Assessment of the second phase of plasma hemostasis - the phase of thrombin formation

Prothrombin (thromboplasty) time. The norm is 11 - 15 seconds. With hypocoagulation, the prothrombin time is increased. With hypercoagulation - reduced.

Prothrombi index, % -

prothrombin time of control plasma.--_-_--_--_-__--_.-----__---_-._-_----„__х 100

patient's prothrombin time________

The norm is 80 - 100% (according to some sources up to 120%).

The norm is from 1 to 1.4.

Assessment of the third stage of blood coagulation

Plasma fibrinogen concentration. The norm is 1.8 - 4.01 g/l. an increase in fibrinogen is observed during hypercoagulation, inflammatory processes,

malignant formations, systemic vasculitis, systemic connective tissue diseases, in the first stage of DIC syndrome. A decrease in the level of fibrinogen can be congenital and acquired (consumptive coagulopathy in DIC syndrome, with primary fibrinolysis).

Thrombin time. The norm is 12 - 16 seconds. Elongation indicates hypercoagulation and lack of fibrinogen in plasma.

ActivityXIIIfactor in plasma. The norm is 70 - 130%. Factor XIII deficiency in C-vitaminosis, leukemia, radiation sickness, severe liver diseases, DIC syndrome with consumption coagulopathy. When factor XIII activity increases, the risk of thrombosis increases.
SCHEME OF EXAMINATION OF PATIENTS History of the disease

1. When clarifying complaints, pay attention to the bleeding that occurs from the mucous membranes during tooth extraction or during teething, during injections (more often in hemophilia and are long-lasting), nosebleeds at night are characteristic of GTB (Werlhof's disease).

2. Pay attention to the nature of skin hemorrhages. In hemorrhagic vasculitis, small punctate petechiae are characteristic, sometimes urticarial and maculopapular elements are located on the extremities, mainly on the extensors, always; symmetrical. In thrombocytopenic purpura, hemorrhages are asymmetrical, preferably localized, polymorphic in nature (from large ecchymoses to petechiae, from purple to blue-green and yellow), in hemophilia they are usually extensive, can be deep with slow resorption and are usually post-traumatic. 3 Pay attention to pain in the joints in hemophilia and hemorrhagic vasculitis. With hemorrhagic vasculitis, there may be arthralgia and swelling of the joints, but they are always reversible, while with hemophilia large joints that have been subjected to trauma are more often affected. The consequence of these lesions can be hemarthrosis.

4. Find out whether there was any previous (3-4 weeks) infectious disease (tonsillitis, scarlet fever, ARVI, etc.), whether vaccinations were carried out, whether food or drug allergies were observed, or whether there were injuries.

5. Clarify whether the child has presented with such complaints for the first time, whether he has been hospitalized before, whether therapy has been carried out, and its results.

Anamnesis of life

1. It is necessary to find out whether bleeding was observed in the patient’s parents and immediate relatives: if the patient is male, then whether the grandfather and father had bleeding.

2. Find out about previous diseases and the presence of chronic foci of infection (chronic tonsillitis, dental caries, tubintoxication, etc.)

Objective research

Determine the patient's condition by severity with an assessment of general development (asthenia, stunting).

When examining organs and systems, first of all, pay attention to:

1. The presence of hemorrhagic manifestations, bleeding from the nose, mucous membranes of the oral cavity, gums, injection sites or skin damage;

2. Skin condition - the presence of asymmetric hemorrhages on the extremities, approximately the same size and shape, or asymmetric hemorrhages of different sizes, arising mainly spontaneously, extensive post-traumatic ecchymosis;

3. The osteoarticular system: the shape of the joints, their mobility, the presence of hemarthrosis and tests;

4. Lymphatic system: involvement of peripheral lymph nodes in the pathological process (with hemorrhagic diathesis they are not involved);

5. Cardiovascular system: the possibility of the appearance of systolic (anemic) noise, more often after bleeding;

6. Respiratory organs (changes are not typical for this pathology);

7. Gastrointestinal tract: presence of abdominal pain, nausea, vomiting, sometimes with blood. Due to severe abdominal pain, the patient takes a forced position on his side with his legs brought to the stomach, rapid stools with blood are possible (abdominal syndrome is characteristic of hemorrhagic vasculitis), the liver and spleen are not enlarged;

8. Renal syndrome: characteristic of hemorrhagic vasculitis (moderate proteinuria with microhematuria), in some cases subacute with transition to chronic glomerulonephritis is observed, bleeding and thrombocytopenia are possible;

9. The presence of metrorrhagia in girls during puberty (with GTB);

10. Changes in the central nervous system: hemorrhagic vasculitis is characterized by transient convulsions and paresis. Hemorrhages in the brain and fundus of the eye are possible.

Based on anamnesis and preliminary data justify a preliminary diagnosis for a specific patient. After substantiating the preliminary diagnosis, outline a plan for examining the patient.

1. General blood test

2. Coagulogram

3. Bleeding time according to Duque

4. Retraction of blood clot5. Biochemical blood tests (fibrinogen, haptoglobin, alpha and gamma globulins, urea, creatinine)

6. Determination of antihemophilic factors (VIII - IX - XI)

7. General urine test

8. X-ray of bones and joints

9. Fundus examination

10. Examination by ENT, dentist, surgeon, orthopedist, neurologist.

Based on the medical history, objective data and laboratory tests, make a clinical diagnosis according to the classification. Specify with what diseases it is necessary to differentiate this disease.

MAKE A TREATMENT PLAN FOR THE PATIENT

3. Replacement therapy (for hemophilia) - transfusion of freshly collected blood, antihemophilic plasma, gamma globulin.

4. To stop bleeding, for local hemostasis, use 5-6% epsilonaminocaproic acid solution, hemostatic sponge, thrombin, gelatin, anterior and posterior tamponade (for bleeding).

5. For bleeding into the joint in the acute period: immobilization, cold, for massive hemorrhage - puncture with aspiration of blood and subsequent administration of hydrocortisone.

6. Non-steroidal anti-inflammatory drugs (indomethacin) - for articular and abdominal syndrome of Henoch-Schönlein disease.

7. Short-course corticosteroid therapy for hemorrhagic vasculitis (with fulminant forms and necrotic variants), and for thrombocytopenia.

8. Heparin therapy for hemorrhagic vasculitis. i - -

9. Transfer to the surgical department for splenectomy (for ITP).

Disturbances in the hemostasis system can affect all its links: vascular, platelet, coagulation (plasma), therefore it is customary to distinguish 3 groups of hemorrhagic diathesis:

1. coagulopathies

2. thrombocytopenia and thrombocytopathy

3. vasopathy

HEMORRHAGIC VASCULITIS (immune microthrombovasculitis, Henoch-Schönlein disease)

One of the most common hemorrhagic diseases (23-25 ​​cases per 10,000 children under 14 years of age) which is based on aseptic inflammation i disorganization of the walls of microvessels, multiple microthrombosis affecting the vessels of the skin and internal organs.

etiology

Unknown. There may be a connection with streptococcal and viral infections, pneumonia, food and drug allergies, burns, hypothermia, etc. In approximately 40% of patients, no specific factor can be identified.

PATHOGENESIS

The pathogenesis consists in the damaging effect on microvessels of circulating immune complexes (CIC) and activated components of the complement system. In a healthy body, immune complexes are eliminated from the body by phagocytic cells. Excessive accumulation of CECs under conditions of predominance of antigens (AG) or with insufficient antibody formation leads to their deposition on the endothelium of the microvasculature with secondary activation of complement proteins along the classical pathway and secondary disorganization of the vascular wall. As a result, microthrombovasculitis develops and the following changes occur in the hemostatic system:

1. Significant activation of platelets, frequent circulation of spontaneous aggregates in the blood.

2. Severe hypercoagulation, combined with a decrease in plasma antithrombin III. which leads to a secondary thrombophilic state and increased heparin resistance.

3. Thrombopenia.

4. Increasing the level of von Willebrand factor. reflecting the severity and extent of damage to the vascular endothelium.

5. Depression of fibrinolysis.

Thus, the formation of platelets and the synthesis of procoagulants during GE exceeds their consumption, which is documented by stable hypercoagulation i hyperfibrinogenemia.

Clinical signs of bleeding - intestinal bleeding, hematuria are a consequence of necrotic changes, reorganization of the vascular wall, and hi thrombocytopenia and consumption coagulopathy (as in DIC syndrome). features should be taken into account when treating patients with hepatitis B.

CLASSIFICATION

(A.S. Kalinichenko, 1996 modified by G.A. Lyskin et al., 2000)

1. Clinical forms (syndromes)

Cutaneous and cutaneous-articular

Simple

Necrotic

With cold urticaria and swelling

Abdominal and cutaneous-abdominal

Renal and cutaneous-renal (including with nephrotic syndrome)

Mixed2. Flow options

Fulminant (in children under 5 years of age)

Acute (allowed within 1 month)

Subacute (allowed up to 3 months)

Prolonged (allowed up to 6 months)

Chronic

3. Activity level:

I degree (minimal) - The condition is satisfactory. The temperature is normal or low-grade. Skin rashes are not abundant. Articular manifestations in the form of arthralgia. Abdominal and renal syndromes are absent. ESR up to 20mm/h

II degree (moderate) - Moderate condition. Severe skin syndrome, fever, headache, weakness, myalgia. Articular syndrome is pronounced. Moderate abdominal and urinary syndrome. In the blood there is moderate leukocytosis and neutrophilia (up to 10x10 /l), eosinophilia, increased ESR - 20-40 mm/h, dysproteinemia, increased gamma globulin content, decreased albumin content.

III degree (maximum) - The condition is severe. Symptoms of intoxication, high temperature, skin syndrome (confluent rash, often with foci of necrosis), articular, abdominal syndrome (paroxysmal abdominal pain, vomiting, mixed with blood) are expressed.

Severe renal syndrome

There may be damage to the central nervous system and peripheral nervous system. Blood: pronounced leukocytosis (10-20x10 9 /l) with neutrophilia, significantly increased ESR (over 40 mm/h), dysproteinemia, may be anemia, decreased platelets.

Complications:

Intestinal obstruction, intestinal perforation, gastrointestinal bleeding, peritonitis, disseminated intravascular coagulation syndrome, posthemorrhagic anemia, thrombosis and infarction in organs.

CLINIC

1. Skin syndrome: papular-hemorrhagic rash against the background of inflammatory infiltration and edema, clearly limited elements of the rash, rarely merging, necrotizing, symmetrical in location, leaving behind brown pigmentation.

2. Joint syndrome: occurs together with the skin. Swelling of large joints and pain of a volatile nature are characteristic. The syndrome quickly resolves, and with relapses, rashes appear.

3. Abdominal syndrome: short-term (no more than 2 - 3 days). Severe course possible: nausea, vomiting with severe abdominal pain, with signs 10

hemocolitis with the development of complications (especially in young children): perforation, intussusception, peritonitis, gastrointestinal bleeding

4. Kidney syndrome: occurs in 1/3 - 1/2 of patients. Develops 1-4 weeks after the onset of the disease. It proceeds according to the CGN type with micro- and macrohematuria. Clinical signs disappear after a few weeks or months.

5. Vascular syndrome: affects the lungs and blood vessels of the central nervous system. In the clinic - headaches, meningeal symptoms. Changes in blood tests - increased fibrinogen, alpha-2 and gamma globulin, von Willebrand factor. Sometimes there may be leukocytosis. With blood loss - anemia, reticulocytosis.

TREATMENT

Diet excluding allergenic foods

Strict bed rest for at least 3 weeks

The administration of fibrinogen, cryoprecipitate, dried plasma and all protease inhibitors, especially epsilonaminocaproic acid, for hemostatic purposes is strictly contraindicated. These drugs enhance the thrombogenic shift, causing depression of fibrinolysis, induce renal thrombosis and cause death of patients.

The use of glucocorticoids is currently considered inappropriate, since it does not shorten the course of the disease and does not prevent kidney damage. Glucocorticoids significantly enhance hypercoagulation, causing depression of fibrinolysis. Prednisolone is indicated for: fulminant forms and necrotic forms

BASIC THERAPY

1. Disaggregants. Curantil suppresses the first wave of aggregation - a dose of 2-4 mg/kg body weight. Trental - orally or intravenously. Indomethacin - has a disaggregation effect - dose 2-4 mg/kg.

2. Heparin- anticoagulant - dose 200 - 700 U per kg of body weight per day subcutaneously or intravenously, frequency of administration at least 4 times a day under the control of blood clotting (according to Lee-White). Discontinuation of the drug should occur gradually with a decrease in the single dose every 2-3 days while maintaining the frequency of administration. If the maximum dose of heparin does not produce an effect, staged plasmapheresis is performed with transfusion of fresh frozen plasma. In severe forms of the disease, especially in fulminant forms, therapy begins with intensive plasmapheresis. The first 3 - 4 sessions daily, then with a break of 1-3 days. Antiplatelet agents and heparin are used in parallel.

3. Fibrinolysis activators. Nicotinic acid and its derivatives (teonicol, complamin).

PREVENTION

Sanitation of foci of chronic infection, clinical observation. Active sports, various physical procedures and stay are contraindicated

in the sun. eleven

HEMOPHILIA

Hemophilia is a hereditary coagulopathy caused by disorders in the blood coagulation system, associated with deficiency or abnormalities of plasma coagulation factors.

Hemophilia affects only men; the disease is caused by damage to a gene located on the X chromosome that controls the synthesis of antihemophilic globulin A (factor VIILC). Hemophilia is transmitted recessively. Women are the conductors (transmitters) of the disease. If a man has hemophilia, and therefore has an abnormal X chromosome and a normal Y chromosome, and a healthy woman with normal X chromosomes. When girls are born, they will all become carriers of hemophilia because they have inherited one abnormal X chromosome from their father and one healthy X chromosome from their mother. The daughters of these parents themselves will not develop hemophilia, because the genetic defect of one X chromosome is compensated by a second healthy X chromosome. The sons of these parents will not have hemophilia and will not pass it on to the next generation because they have inherited a healthy Y chromosome from their father and a healthy X chromosome from their mother.

Thus, of all the children of a man with hemophilia, the sons will be 100% likely to be healthy, and the daughters will be 100% likely to be carriers (conductors) of hemophilia. Women who carry the hemophilia gene do not have clinical manifestations of hemophilia, but can give birth to sons with hemophilia. If a woman who is a carrier of hemophilia and has one healthy and one abnormal X chromosome marries a healthy man, then her sons can be either healthy or have hemophilia, and her daughters can be either healthy or carriers of the hemophilia gene. Consequently, the sons of female carriers of hemophilia have equal chances of receiving a pathological or normal X chromosome, i.e. 50% will be born with hemophilia. Daughters of female carriers have a 50% risk of being a carrier of the hemophilia gene. Women who are carriers (conductors) of the hemophilia gene have a second normal X chromosome and, as a rule, do not suffer from bleeding; the coagulant activity of antihemophilic globulin (UPG.C factor) is reduced on average by half and is about 50% of the norm.

In rare cases, hemophilia in girls is possible if they inherit 2 atypical X chromosomes: one from a father with hemophilia, the other from a mother who is a carrier of hemophilia.

The most characteristic clinical symptom with hemophilia there is bleeding, which has the following features:

1. Bleeding is excessive compared to the cause that caused it;

2. Bleeding in hemophilia is prolonged, lasting for hours and can persist for several days;

3. Bleeding in hemophilia does not occur immediately after injury, but after two hours. The clot that forms at the site of injury is loose, wide, and voluminous, but does not help stop the bleeding, since blood continues to ooze along its edges.

4. Haemophilus influenzae bleeding tends to recur where there was bleeding before.

5. Bleeding in hemophilia tends to spread; hematomas are often formed, which can penetrate into muscles, joints and internal cavities.

A person with hemophilia bleeds frequently, easily, for a long time and profusely. When the integrity of the capillaries is violated, it occurs in any damaged area of ​​the body. Patients with hemophilia are no different in appearance from healthy children. They become pale only after blood loss. When hemarthrosis appears, local

muscle atrophy. With the development of secondary anemia, a systolic murmur appears at the apex and a slight expansion of the boundaries of cardiac dullness.

The gastrointestinal tract is unremarkable, the liver and spleen are not enlarged.

The urinary system, if there is no hematuria and stones, is without any peculiarities.

Neurological studies reveal changes only in cases of compression of nerves by hematomas. With cerebral hemorrhages, neurological symptoms depend on the location of the hemorrhage.

Clinically, in hemophilia, the following types of hemorrhage are distinguished:

Subcutaneous hemorrhages

Skin bleeding

Bleeding from mucous membranes

Hematomas and hemorrhages in the central nervous system

Bleeding in the joints (hemarthrosis)

According to the clinical course, hemophilia is divided into three forms:

* moderate severity

* heavy: ,

The most important laboratory tests are:

1. Indicators of delayed venous blood coagulation;

2. Indicators of decreased activity of coagulation factors VIII and IX

3. Indicators of reduction in prothrombin consumption

Currently, it is important not only to diagnose hemophilia, but also to establish the form of hemophilia in a given patient: hemophilia A or B. With hemophilia A, there is a lack of labile antihemophilic globulin (AGG) in the patient’s blood; with hemophilia B, there is a deficiency of a more stable component - plasma thromboplastin (KTP). AGG is consumed during blood clotting, and CTP acts catalytically.

With the aim of differential diagnosis of hemophilia A and B The following additional laboratory tests are used:

1. Mixing the blood plasma of patients with hemophilia A and B normalizes the clotting time of recalcified oxalate plasma.

2. Addition of AGG to the test plasma causes normalization of coagulation of recalcified oxalate plasma in hemophilia A, but does not affect plasma coagulation in hemophilia B.

3. Adding stale serum from a healthy person to the plasma of a hemophilia patient normalizes the coagulation of recalcified oxalate plasma in hemophilia B; it is not effective in hemophilia A, since the stale serum contains CTP and contains AGG. 13

The pathogenesis of bleeding in hemophilia is complex. Here there is damage to the hemostatic system, depending on blood coagulation disorders and functional damage to blood vessels. Coagulopathy in hemophilia is caused by a slowdown in the formation of active thrombinase due to a lack of AGG or CTP in the blood plasma. Increased platelet resistance is of some importance. In hemophilia, protein metabolism is disrupted. Changes in enzymatic and mineral metabolism, as well as endocrine-vegetative shifts, are noted. The pathological predominance of androgenic sex hormones over estrogenic hormones slows down blood clotting.

DIFFERENTIAL DIAGNOSIS OF HEMOPHILIA carried out with all congenital hemorrhagic diathesis:

1. hypothromboplastinemia (von Willebrand-Jurgens, congenital deficiency of Hageman factor)

2. hypothrombinemia

An accurate diagnosis is made by examining all coagulation factors; with inhibitory hemophilia, a positive reaction to the presence of positive anticoagulants.

TREATMENT OF HEMOPHILIA

All external bleeding in hemophilia is treated locally. To remove clots, the wound is washed with penicillin diluted with saline. Then gauze soaked in one of the hemostatic agents (adrenaline, thromboplastin-rich hemostatic sponges) is applied. Tampons with fresh human milk are good for bleeding from the oral cavity and nasal mucosa. Cow's milk does not have this effect because it does not contain sufficient thromboplastin. We must remember that a bleeding wound must be well compressed and packed.

If possible, the wound should not be stitched. If the bleeding does not stop under the influence of local treatment, the hemostatic effect should be achieved with general treatment.

The first place among the general methods of treating bleeding in patients with hemophilia is blood transfusion. The hemostatic effect of blood transfusions occurs due to:

1. Large amounts of AGG and CTP in transfused blood

2. The beneficial effect of transfused blood on the capillaries, the walls of which become denser. In addition, blood transfusions stimulate the bone marrow and replace blood loss.

At HEMOPHILIA And fresh blood rich in labile AGG should be transfused

(factor VIII), and when GSMOPHILIA V- ordinary donor, “stale” blood, since the latter contains a stable component of plasma thromboplastin - CTP (factor IX) in sufficient quantities. 14

If it is impossible to determine the type of hemophilia, one should prefer

transfusion SVSZH6Y blood or plasma (considering that most patients with hemophilia are type A).

The number of required transfusions varies among patients with hemophilia. It depends on the level of factors VIII and IX in the blood of patients and the blood of the donor. Bleeding stops when the level of factors VIII and IX reaches 25 - 30%. In cases of large blood loss, an infusion of large doses of blood is taken: for younger children - 5 - 10 ml / kg, for older children a single dose - 150 - 2000 ml.

Recently, a drug enriched with AGG, cryoprecipitate globulin, has been prepared. The concentration of antihemophilic globulin is 15 to 20 times higher than its concentration in normal plasma.

The English scientist Brinkhouse obtained a cryoprecipitate in which the concentration of AGG is 100 times higher than its concentration in normal plasma. Old and fresh human serum for hemophilia B and C in a dose of 20 ml under the skin has a good hemostatic effect.

For the purpose of long-term prevention of bleeding in hemophilia B, 20 ml of human serum should be injected under the skin every month for a year. Then every 2 months in the same dosage.

Drugs that reduce the permeability of the vascular wall are widely used: calcium chloride, lactic acid, calcium phosphate, calcium gluconate.

The use of vitamin K in hemophilia does not give satisfactory results, since vitamin K increases the prothrombin level in the blood, while in hemophilia the amount of prothrombin is normal.

Vitamin P acts mainly on vascular permeability and does not occupy a dominant place in the treatment of hemophilia.

Surgery in patients with hemophilia may be necessary for diseases not associated with hemophilia, when treating complications of hemophilia. When there are vital indications for surgery (strangulated hernia, acute appendicitis, etc.), it should be performed painlessly. 1 hour before surgery, a transfusion of fresh blood is done for hemophilia A and regular donor blood for hemophilia B.

Transfusion is repeated 12 hours after surgery. For abdominal operations, general anesthesia should be used. Treatment is carried out according to general surgical rules.

Despite advances in the treatment of hemophilia, the prognosis remains serious, especially in children.

HEMORRHAGIC THROMBOCYTOPENIC DISEASE GTB is a general disease of the body involving many regulatory mechanisms in the pathological process. Damage to the hemostatic system is only a partial expression of this. The essence of the process is the disruption of the formation or “detachment” of platelets from megakaryocytes.

The disease can be detected at any age, even during the neonatal period, although it most often occurs in children 5-6 years old.

In the etiology and pathogenesis of GTB, dysfunction of the nervous system, autonomic endocrine system, reticuloendothelial system, and metabolic changes are important. The main pathogenetic factors of impaired 1

" " " 15

hemostasis are changes in the vascular wall, thrombocytopenia and associated physicochemical blood disorders.

Clinical classification involves dividing GTB into three forms: light, medium and heavy. According to the course of the disease they distinguish acute, acute and

chronic form. "=." "..-,

CLASSIFICATION OF HEMORRHAGIC DIATHESES IN CHILDREN

Thrombocytopenic purpura according to A.B. Mazurin, 1996.

Type: L. Congenital

B. Acquired Form:! non-immune:

II autoimmune -

III isoimmune ":

IV medicinal (allergic) Period: 1. Crisis in severity: a) mild

b) medium-heavy

c) heavy

2. Clinical remission

3. Clinical and hematological remission Course: 1. Acute

2. Chronic: a) with rare relapses b) with frequent relapses ____________c) continuously relapsing

hemorrhagic

vasculitis according to A.S. Kalinichenko, 1996

According to clinical manifestations: simple. (skin lesions) and mixed (articular, abdominal and renal syndrome) By types and variants of the course: "" .

A) spicy, ? B) subacute (protracted)

B) chronic

D) recurrent

OUTCOMES: 1. Recovery

2. Transition to chronic form

3. Outcome to chronic nephritis

Hemorrhagic thrombocytopenic disease clinically manifested by subcutaneous and skin hemorrhages, spontaneous bleeding from the mucous membranes due to damage to blood vessels and a sharp decrease in the number of platelets in the blood. With this disease, the duration of bleeding is increased, the retraction of the blood clot is absent, and capillary resistance is reduced. The disease occurs in children of both sexes. An objective examination of children with GTB shows decreased nutrition and pale skin. On auscultation, a systolic murmur is heard at the apex of the heart. The spleen can be felt below the costal arch. Otherwise, there are no deviations from the internal organs. Subcutaneous hemorrhages in GTB are characterized by:

1. Polymorphism: Along with large echimosis, a small petechial rash is detected.

2. Polychrome: bright red, blue, greenish, yellow colors.

3. Various localizations: skin, mucous membrane of the palate, tonsils, pharynx, posterior pharyngeal wall.

Hair follicles are not affected and are free from hemorrhages, which differs from scurvy. 16

Subcutaneous hemorrhages are such a common symptom that in their absence the diagnosis of hemorrhagic thrombocytopenic purpura is usually incorrect. With hemorrhagic thrombocytopenia, there is no tendency to spread subcutaneous hemorrhages, so there is no blood depot under the skin, therefore, suppuration and nerve paresis rarely occur.

Cavitary hemorrhages in children include hemorrhage in the oral cavity, nose, and bleeding from the socket of an extracted tooth. Rarely there are hemorrhages in the eye area, bleeding from the ears, and hematuria is rarely observed. Cerebral hemorrhages are possible, which develop during the course of the disease, and may be the first early signs of it. Skin bleeding is not uncommon; it can be prolonged, but is not as threatening as in severe hemophilia.

Hemarthrosis and hematomas are rare. The diagnosis is made on the basis of anamnesis, clinic and laboratory tests.

LABORATORY SIGNS

1. A characteristic feature of hemorrhagic thrombocytopenic disease is a decrease in the number of platelets in the peripheral blood. Normally, in children, the platelet count is 300,000 in I mm j (A.F. Tur). With GTB, in one group of children the number of platelets is reduced slightly and ranges from 80,000 - 100,000, in others it is sharply reduced - to 20,000 - 30,000, in others it reaches 10,000 and below. . ...

2. The duration of bleeding increases. Normally, the duration of bleeding is 2.5 - 3 minutes (according to Duque). With GTB, the duration of bleeding reaches 15-30. -minutes, and in some cases several hours. The duration of bleeding depends on the reduced resistance of the capillaries and the disturbance of the contractile reaction of the blood vessels.

3. Blood clot retraction - significantly reduced or completely absent. Normally, the retraction index is 0.3-0.5. .-" . h "

4. Determining the degree of resistance and fragility of capillaries is of great diagnostic importance. In GTB, the tourniquet symptom is sharply positive.

5. Blood clotting time is usually normal. " - . ;

6. The prothrombin level is normal and the prothrombin indicator is 83 - 100%.

7. The amount of fibrinogen in the blood is normal. .

8. Reticulocytosis during bleeding is well expressed. The number of reticulocytes increases to 20-40% 0 , in isolated cases reaching 100% 0 .

Necessary differentiate GTB with illness Schönlein-Henoch in which hemorrhages are localized in the area of ​​large joints and on the buttocks.

Unlike GTB, with hemorrhagic vasculitis there is swelling and tenderness of the joints, cramping abdominal pain and diffuse hemorrhagic

Nephritis; bleeding from the mucous membranes is not prolonged, therefore, secondary anemia does not develop in these patients, the spleen is not enlarged. Laboratory findings are contrary to those obtained in thrombocytopenic disease.

Platelet count, duration of bleeding and clot retraction

Fine. Differential diagnosis with hemophilia described in the Hemophilia section.17

When diagnosing scurvy, it is necessary to take into account that hemorrhages in the latter are localized around the hair follicles, which is not the case with GTB. In both diseases, hemorrhages occur in the gum area. With GTB they are located on healthy mucosa, and with scurvy - on inflamed mucosa. The amount of ascorbic acid in the blood during scurvy is sharply reduced.

Carrying out differential diagnosis with pseudohemophilias, It must be remembered that with the latter, the content of factors I, II, V or VII in the blood is reduced. In GTB, the content of coagulation factors is normal. In patients leukemia Hemorrhagic phenomena and thrombocytopenia appear early. The difference is the pronounced hepato-lienal syndrome. The presence of young forms of white blood in the blood, progressive anemia and a more severe course with leukemia.

To fully cover the hemorrhagic syndrome, it is necessary to identify diseases that occur with disorders of the reticuloendothelial system, metabolic disease, cardiovascular system, and, accompanied by hemorrhages to varying degrees.

In the clinic of most liver diseases, especially severe ones (viral hepatitis, cirrhosis, acute dystrophy), hemorrhagic syndrome appears. Active coagulation proteins are formed intra- and extrahepatically, while damage to the liver parenchyma leads to a decrease in plasma factors I, II, V, VII, IX, X.

At glycogenic hepatosis hemorrhages are caused by the lack of glucose-6-phosphatase in platelets.

In kidney diseases, hemorrhages are less common; they can be found in 1/3 of patients with acute and chronic uremia. Uremia is characterized by hemorrhages of the meninges, endocardium, pericardium, and pleura.

In patients.s congenital heart defects, especially with a left-right shunt is detected. tendency to hemorrhages, congestive liver, oxygen deficiency of the bone marrow and liver - chronic hypoxia, reactive erythrocytosis, which contribute to the appearance of significant disturbances in the process; blood clotting.

Clinically, patients with congenital heart defects develop diffuse, spotty hemorrhages in the skin and mucous membranes, and less often, bleeding from the upper respiratory tract and gastrointestinal tract.

In every case of hemorrhagic syndrome in children, one should think about acute leukemia! "

Main indicators of a normal coagulogram (according to E. Ivanov, 4983)

1.Childhood diseases. Edited by L.A. Isaeva, 1996

2.Childhood diseases.Edited by N.P.Shabalov, 2002

Additional "

1. M. P. Pavlova Hematological diseases in children. Minsk, 1996

2.I.A. Alekseev Pediatric hematology St. Petersburg, 1998

3.B.Ya.Reznik Pediatric hematology with atlas of myelograms Kyiv, G

Hemorrhagic diathesis is a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and manifested by an increased tendency to bleeding and hemorrhage.

Etiology

The heredity of hemorrhagic conditions is determined by abnormalities of megakaryocytes and platelets, defects in plasma coagulation factors, and inferiority of cervical blood vessels.

Acquired hemorrhagic diathesis is caused by disseminated intravascular coagulation syndrome, toxic-infectious conditions, liver diseases, and the effects of drugs.

Classification

1. A disease caused by a violation of vascular hemostasis (vasopathy).

1) Shenein-Henoch disease (simple, rheumatoid, abdurate and fulminant purpura);

2) hereditary familial simple purpura (Davis);

3) anular telangiectatic purpura of Mabocca;

4) necrotic Sheldon's purpura;

5) hyperglobulinemic Waldenström's purpura;

6) hereditary hemorrhagic telangiectasia;

7) Louis-Barr syndrome (capillary telangiectasia of the conjunctiva with ataxia and chronic pneumonia);

8) Kasabach-Merritt syndrome;

9) scurvy and Mimer-Barny disease;

2. Diseases caused by a violation of the platelet mechanism of hemostasis (thrombocytopathy, thrombocytopenia):

1) hemorrhagic thrombocytopathy, Werlhof's disease;

2) amegakaryocytic thrombocytopenic purpura of Landolt;

3) autoimmune thrombocytopenia of various origins;

4) thrombocytopenic hymphragic purpura with acquired autoimmune hemolytic anemia (Evens-Fisher syndrome);

5) thrombocytopenic purpura with a chronic purulent shade and exudative diathesis (Ondrich syndrome);

6) thrombotic thrombocytopenic purpura of Mogmkovitsa;

7) thrombocytopenia in geangiomas (Kasabach-Merritt syndrome);

8) hereditary properties of thrombopathy (Glanuman, Willibrand);

9) thrombocytopathy in combination with a violation of coagulation factors.

3. Diseases caused by disorders of blood clotting factors (coagulopathy):

1) hemophilia A due to deficiency of factor VIII;

2) hemophilia B due to deficiency of factor IX;

3) hemophilia C due to deficiency of factor XI;

4) pseudohemophilia due to hypoprothrombinemia;

5) Ouren's pseudohemophilia;

6) pseudohemophilia due to deficiency of factor VII;

7) pseudohemophilia due to a lack of fibrinogen (afibrinogenemia);

8) pseudohemophilia due to lack of factor X;

9) pseudohemophilia due to lack of fabrinase;

10) pseudohemophilia due to excess anticoagulants.

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  Diathesis hemorrhagic. Hemorrhagic diathesis


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  Diathesis hemorrhagic. Hemorrhagic diathesis– a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and...


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  Diathesis hemorrhagic. Hemorrhagic diathesis– a group of diseases characterized by impaired hemostasis (vascular, platelet or plasma) and...


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  Diathesis hemorrhagic. Hemorrhagic diathesis


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  Diathesis hemorrhagic. Hemorrhagic diathesis– a group of diseases characterized by impaired hemostasis (vascular.


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  Diathesis hemorrhagic. Hemorrhagic diathesis– a group of diseases characterized by impaired hemostasis (vascular.