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Isoimmune Werlhof's disease - 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 isoimmune Werlhof's disease is observed at the newborns who were born from healthy mothers. The pathogeny of a disease is similar to a hemolitic illness of newborns and is explained by incompatibility of a fruit and mother on platelet antigens. In most cases the immunological havoc is caused by existence at a fruit of platelet antigen of PLA1, as a result of isoimmunization in an organism of mother the antithrombocytic antibodies directed against antigen of thrombocytes of the child are developed. As a rule, an antiserum capacity high that leads to emergence of a Werlhof's disease of newborns already at the first pregnancy of mother. It is known that at a Rhesus factor conflict the hemolitic illness of newborns at primapara women develops rather seldom. Except PLAl antigen isoimmunization can be caused also by other platelet antigens or antigens, the general for thrombocytes and leukocytes (Duzo, HLA — 5, HLA — 9). The isoimmune Werlhof's disease of newborns meets seldom — 1 case on 5000 — 10 000 newborns.
The isoimmune Werlhof's disease is clinically characterized by an acute current. Already during the first hours after the child's birth the quantity of thrombocytes decreases to 10 — 50 X 109/l and plentiful hemorrhages — petechias and ecchymomas with preferential localization on a trunk appear. Plentiful bleedings from mucous membranes of nasal and oral cavities, from the umbilical rest, gastric bleedings can be observed. The cephalohematomas and hematencephalons which are the reason of a lethal outcome are possible. According to different authors, mortality at an isoimmune Werlhof's disease of newborns makes 12 — 14%. However the disease can proceed also in not severe form when in a clinical picture only skin petekhialny hemorrhages are observed, bleedings are absent, and the quantity of thrombocytes is normalized within several days. Possibly, weight of an isoimmune Werlhof's disease of newborns depends on a caption of antithrombocytic antibodies. Laboratory at newborns in peripheral blood define various degree of thrombocytopenia.
At immunization antigens, by the general for thrombocytes and leukocytes, along with thrombocytopenia observe a leukopenia. In most cases the forecast at an isoimmune Werlhof's disease of newborns favorable. Under the influence of therapy within several days it is possible to stop a hemorrhagic syndrome. For 2 — 10 weeks also the quantity of thrombocytes is normalized.
In the absence of bleedings it is possible to be limited to purpose of sosudoukreplyayushchy means (drugs of calcium, Rutinum, Dicynonum, aminocapronic acid). Important therapeutic action is otnyaty the child from mother's breast as women's milk contains antithrombocytic antibodies. Existence of bleedings or a "wet" form of skin purpura are indications for purpose of Prednisolonum in a daily dose of 2 mg/kg of body weight. At considerable thrombocytopenia and not stopped bleeding appoint transfusion of a platelet concentrate (to 50 ml).
In literature cases of the isoimmune Werlhof's disease which developed after hemotransfusions are described. The pathogeny of this disease is difficult. Assume that at introduction of the thrombocytes containing PLAl antigen , to persons who are deprived of this antigen there is a development of antibodies. The further mechanism is similar to heteroimmune process. The antigen (donor thrombocytes) formed a complex — an antibody is fixed on the patient's thrombocytes that leads to development of thrombocytopenia. The available supervision concern adults, in pediatric practice of similar cases is not described.



 
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