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Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis - 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

Hereditary elliptotsitoz (ovalocytosis).

Erythrocytes of an oval form phylogenetic more ancient, than round. They are accessory of the lowest vertebrata, and among mammals are observed at camels. Adults and for children also have an oval form erythrocytes, however quantity their small (from 1 to 10%) in this connection they do not define a possibility of an increased hemolysis. Speak about an elliptotsitoza when the number of dipsotherapies exceeds 25%. At some persons the quantity of erythrocytes of an oval form can make 80 — 90%. In genesis of a disease assume defect of a membrane of erythrocytes. Disturbance of transport of ions of sodium is proved (S. Roberts and soavt., 1966). The disease is descended, a mode of inheritance autosomal and dominant. At most of persons clinical displays of a disease are absent, and it can be diagnosed
at accidental research of peripheral blood. According to literature, only at 12% of persons with oval erythrocytes the increased hemolysis leading to development of anemia can be observed. Clinically the ovalocytosis proceeds in the form of not heavy hemolitic process with moderate pallor of skin and an ikterichnost of scleras. However in literature children have data on heavy hemolitic processes 1 — 2-month age. In blood define moderate anemia with good regenerator reaction (a high reticulocytosis). Reticulocytes have the round form.
At an ovalocytosis trophic ulcers of a shin, and also radiological changes of bones of a skull are described. In the USSR this form of a disease is described by Yu. I. Loriye (1963) and other authors. Now it is shown what elliptotsitoz is not such rare pathology and frequency makes it 2 — 4 cases on 10 000 population, that is same as hereditary spherocytosis.
In the absence of hemolitic process of treatment it is not required. If the disease is followed by hemolitic anemia, an effective method of therapy is the splenectomy.

Hereditary stomatocytosis

Hereditary stomatocytosis — extremely rare form of morphological inferiority of erythrocytes which is combined with hemolitic anemia. The term "stomatocytosis" was entered by S. P. Lock and coauthors (1961). The stomatocytosis is inherited on autosomal dominantly type. Assume that the hereditary defect of proteins of a membrane of erythrocytes which is characterized by increase in membrane permeability for sodium ions is the cornerstone of a disease (L. I. Idelson, 1979). At morphological studying of erythrocytes the following feature is noted: their central part is more pale, than all erythrocyte, has the linear, but not round, as usual, form reminding a rotoobrazny opening in a cell.
At many patients the stomatocytosis proceeds asymptomatically. In the presence of hemolysis the clinical picture reminds a hereditary spherocytosis — moderate jaundice, pallor, increase in the sizes of a spleen.
In peripheral blood — anemia with the raised reticulocytosis. Diagnosis is based on detection in blood of stomatocytes.
At the asymptomatic course of treatment it is not required. In the cases proceeding with hemolitic crises the splenectomy which, however, is less effective, than at a hereditary spherocytosis is shown.



 
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