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Trombogemolitichesky Werlhof's disease (a syndrome Moshkovich) - Practical hematology of children's age

Table of contents
Practical hematology of children's age
Embryonal hemopoiesis
Morfofunktsionalny characteristic of cells of marrow and peripheral blood
Marrow parenchyma cells
Peripheral blood of children of different age
The system of a hemostasis is normal
Etiology and pathogeny of leukoses
Acute leukoses
Acute leukoses - a preleukosis
Possibilities of a predictive assessment of a course of an acute lymphoblastoid leukosis at children
General principles of treatment of an acute leukosis
Chemotherapeutic drugs
Treatment of an acute lymphoblastoid leukosis
Treatment of myeloid forms of an acute leukosis
Infectious complications and symptomatic therapy of an acute leukosis
Consolidation and maintenance therapy of an acute leukosis
Immunotherapy
Remission and recurrence of an acute leukosis
Inborn leukosis
Neuroleukosis
Myelosis
Lymphogranulomatosis
Gematosarkoma
Macrofollicular lymphoma
Angioimmunoblastny lymphadenopathy
Leukemoid tests
Infectious lymphocytosis
Infectious mononucleosis
Leukemoid tests of different types
Dysfunctions of granulocytes
Leukopenias
Histiocytoses
Histiocytoses - an eosinophilic granuloma
Malignant histiocytosis
Family erythrophagocytal histiocytosis
Accumulation diseases
Nimann's illness — Peak
Angiopathies
Hemorrhagic vasculitis (Shenleyn's illness — Genokh)
Mayokki's purpura
Ataxy teleangiectasia
Entsefalotrigeminalny angiomatosis
Kortiko-meningealny diffusion angiomatosis
Cerebroretinal angiomatosis
Hypertrophic gemangiektaziya
Multiple and huge hemangiomas
Elastic fibrodisplaziya
Coagulopathies
Hereditary coagulopathies
Hemophilia And
Clinic of hemophilia
Treatment of hemophilia
Angiohemophilia
Cristmas disease (Kristmas's illness)
Hereditary deficit of factors of XI, XII, XIII and I
Dysfibrinogenemias
Hereditary deficit of factors of VII, X, V and II
Deficit K-vitaminozavisimykh of factors of coagulation
Syndrome of the disseminated intravascular coagulation
Clinic and diagnosis of the IDCS
Treatment of the IDCS
Thrombocytopenia
Idiopathic Werlhof's disease
Clinic and diagnosis of an idiopathic Werlhof's disease
Treatment of an idiopathic Werlhof's disease
Isoimmune Werlhof's disease
Transimmune Werlhof's disease of newborns
Trombogemolitichesky Werlhof's disease (syndrome Moshkovich)
Hereditary Werlhof's diseases
Trobotsitopatiya
Anemias
The anemias connected with blood loss
Chronic posthemorrhagic anemia
Iron deficiency anemias
Clinic and diagnosis of an iron deficiency anemia
Treatment of iron deficiency anemias
Sideroakhrestichesky, sideroblastny anemias
Megaloblastny anemias
Foliyevodefitsitny anemia
Hereditary forms of megaloblastny anemias
Hereditary dizeritropoetichesky anemias
The anemias connected with oppression of proliferation of cells of marrow
Hereditary hypoplastic anemias
Hemolitic anemias
Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis
Acanthocytosis, piknotsitoz
The hereditary hemolitic anemias connected with disturbance of activity of enzymes of erythrocytes
The hereditary hemolitic anemias connected with disturbance of structure or synthesis of hemoglobin
The acquired immune hemolitic anemias
Isoimmune hemolitic anemias
Treatment of a hemolitic illness of newborns
Autoimmune hemolitic anemias
List of references

The Trombogemolitichesky Werlhof's disease (a syndrome Moshkovich) is for the first time described by E. Moschowitz in 1925 under the name of the acute feverish pleyokhromny (hemolitic) anemia which is characterized by formation of hyaline blood clots in terminal arteries and capillaries.
In 1936 R. L. Baeher allocated this disease in separate nosological unit. In literature it is known under different names, more correct, remained till our time, offered in 1954 E. Adelson and coauthors — "a trombogemolitichesky Werlhof's disease".
The etiology is not clear, a number of authors assume the infectious nature. Acute autoagressivny process is the cornerstone of a disease. In blood of patients find anti-erythrocyte, antithrombocytic and anti-endothelial antibodies. Except thrombocytopenia, bleeding raised by one of mechanisms the consumption coagulopathy is.
Pathomorphologic substrate of an illness are the multiple changes in small vessels (capillaries, precapillaries, arterioles) consisting in fibrinoid defeat of walls, their segmented necrosis without the expressed inflammatory reaction and with education in them peculiar hyaline blood clots which part the bigger or smaller quantity of thrombocytes and erythrocytes is. Sometimes find proliferation of an endothelium in the thrombosed vessels (L. A. Makhonova and soavt., 1965).
The syndrome Moshkovich occurs at any age, even at babies, but is generally observed at adults (80%), mainly at women.
The disease is characterized by a combination of three main syndromes: 1) Werlhof's disease; 2) hemolitic anemia; 3) a thrombovasculitis in the form of diffusion defeat of small vessels (arteries, venules and capillaries) with formation of hyaline blood clots. Vessels of a brain, kidneys, spleens, a liver are surprised.
The illness proceeds as an acute feverish disease. Temperature increases, the general weakness, a headache, jaundice, a joint pain, muscles, sometimes in a stomach appear. Along with it the hemorrhagic syndrome which is shown hemorrhages on skin and mucous membranes and also nasal, gastrointestinal, renal, uterine bleedings develops.
Skin is pale, with an icteric shade, small increase in a liver, spleen and lymph nodes is quite often noted. Characteristic symptoms are psychological frustration: concern, drowsiness, block, sometimes a black-out, up to development of a coma. Paresis, paralyzes, disturbance of the speech (aphasia), tonic spasms and even maniacal states are possible. The specified psychological frustration are caused by thrombosing of vessels of a brain. Defeat of vessels of kidneys causes a heavy nephropathy with fast development of a renal failure. Along with it dysfunctions of other bodies in connection with development of multiple thromboses in various sites of a vascular network (lungs, a myocardium, bark of adrenal glands, lymph nodes etc.) are noted.
In blood — anemia with a reticulocytosis, a polychromatophilia, a poikilocytosis and an anisocytosis of erythrocytes, normoblasts and erythroblasts can be also found. Osmotic firmness of erythrocytes is often not changed, the level of an indirect bilirubin is increased. Thrombocytopenia of various degree of manifestation with disturbance of retraction of a blood clot, increase in duration of bleeding is constant. The quantity of leukocytes fluctuates from norm to a moderate leukocytosis, the neutrocytosis with shift is more often noted to the left. A marrowy hemopoiesis is characterized by signs of irritation of an erythroidal sprout inherent to hemolitic process. Also the hyperplasia of a megakariotsitarny row is noted.
The forecast is adverse. The death occurs within several days or weeks from the beginning of a disease owing to a coma, development of renal and heart failure, sometimes from a hematencephalon.
Corticosteroid hormones are ineffective in treatment of a syndrome Moshkovich. The main therapy has to be directed to fight against bleeding and development of the IDCS.
Evans's syndrome — Fischer. R. Evans (1943) and M. is described by Fisher (1947). Represents a thrombocytopenia combination to hemolitic anemia. It arises under the influence of various etiological factors — a viral and bacterial infection, reception of medicines. The pathogeny of a disease is regarded as autoimmune process. In blood of patients find anti-erythrocyte and antithrombocytic antibodies. Degree of manifestation of a hemorrhagic and hemolitic syndrome, and also sequence of their development are not identical. In most cases thrombocytopenia precedes hemolysis development. In a clinical picture the skin hemorrhagic syndrome is noted; bleedings, as a rule, are absent, even at the expressed thrombocytopenia. Further there are symptoms of hemolitic anemia — pallor of skin, its icteric shade, moderate increase in a liver and spleen. At height of a disease the general state is broken, body temperature increases. The picture of blood is characterized by existence of anemia with a reticulocytosis, an anisocytosis and a poikilocytosis of erythrocytes, thrombocytopenia. The forecast at this pathology serious. Drug treatment is carried out according to the schemes accepted in therapy of autoimmune hemolitic anemias and an idiopathic Werlhof's disease.
L. I. Idelson (1979) notes the high frequency of a combination of autoimmune thrombocytopenia to autoimmune hemolitic anemia, and temporary ratios between these two forms often happen unequal. From these positions the author regards them as two various options of a cytopenia which are combined among themselves, but not as the uniform syndrome and suggests to refuse the accepted term "Evans's syndrome — Fischer".



 
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