Hemolitic anemias - an ovalocytosis, a hereditary stomatocytosis - Practical hematology of children's age

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.