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Malignant histiocytosis - 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 malignant histiocytosis is for the first time described in 1939 by R. Century of Scott and A. N. T. of Robb-Smith. The disease is initially tumoral process with defect in system of the englobing macrophages. Mainly adult are ill, is more rare — children of advanced age. Hereditary aspects are not revealed. On clinical and pathomorphologic signs the malignant histiocytosis has a certain similarity to a histiocytosis of X, especially with Letterer's illness — Ziva. Development of a disease is caused by proliferation of macrophagic elements in various fabrics — a liver, a spleen, lymph nodes, skin, marrow. Unlike a histiocytosis of damage of a bone tissue it is not observed. Pathomorphologic substrate is provided by atypical histiocytes, multinucleate cells meet. An important differential diagnostic character is existence in cytoplasm of fagotsitirovanny erythrocytes, leukocytes and thrombocytes. Cells of a malignant histiocytosis differ in the expressed tumoral anaplaziya though studying of markers shows that they belong to histiocytic elements. The disease arises sharply and it is very difficult to catch early symptoms. The condition of patients considerably worsens — appetite disappears, loss in weight progresses. The sharp weakness, high temperature, a dermahemia, papulo-makulezny rash sometimes accepting hemorrhagic character, necroses of skin and mucous membranes are noted. The considerable gepatosplenomegaliya is typical; bodies at a palpation dense, and at fast increase — are painful. Damage of a liver can lead to development of jaundice and ascites further. At many patients lymph nodes increase. The hyperplasia is moderate, nodes dense, painless. Specific pulmonary and cordial changes are shown by exudative pleurisy and a pericardis. In blood — increase in SOE, a leukocytosis. Anemia is constantly noted. Development of anemia is caused both by damage of marrow, and a hemolitic component. The quantity of reticulocytes is increased, normocytes meet. In a late stage of a disease the pancytopenia develops. In some cases at blood there are atypical histiocytes. In marrow — the progressing infiltration histiocytic elements with phagocytosis signs. Differential diagnosis needs to be carried out with a histiocytosis of X. The malignant histiocytosis distinguishes lack of bone destructions and existence of the anaplazirovanny histiocytes englobing erythrocytes, leukocytes and thrombocytes. The disease is characterized by a rapid malignant current. The forecast is adverse. Carry out complex treatment: corticosteroids in combination with cytostatic drugs (Vincristinum, 6 Mercaptopurinum, Cyclophosphanum); symptomatic means, antibiotics, transfusions of components of blood, disintoxication therapy.



 
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