Thalassemia

Thalassemia is disorder of normal hemoglobin synthesis. Its changed molecules transport oxygen worse. Red bloody little bodies with the changed hemoglobin are unstable and quicker perish.

Illness is often met in Europe, Transcaucasia and Central Asia. If the person inherited defective genes from both parents, implications of illness are much more serious.

The clinic of thalassemia is shown in the childhood. Sick children have a peculiar “tower” skull, wide Mongoloid face, enlarged top jaw. In patients liver and spleen are enlarged. In some time cirrhosis, diabetes mellitus, congestive heart failure are formed. All this is connected to strengthened hemopoiesis and destruction of erythrocytes, accumulation and sediment of iron compounds in the internals. The child has development gap, skin is cyanotic and icteric, there are trophic ulcers on sheens. Often gallstone disease illness develops, bilirubin stones are found in the cholic courses.

Thalassemia is treated with transfusion of erythrocytes. The packed red cells at frequent transfusions is more preferable than an integral blood, it contains less antigens irritating immune system.

At a serious form of illness the need for transfusions arises from first months of child life and remains for life. Hemoglobin level in blood of patients decreases without transfusions, and there are no other ways of its increasing. It is desirable to carry out transfusion at hemoglobin level of 95-100 g/l, without allowing it to decrease.

Selection of donors is carried out individually as at continuous transfusions the immune system starts reacting with other proteins of donor’s erythrocytes surface besides antigens of systems AVO and rhesus which are always considered.

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