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Thalassemia

Thalassemia (synonym: Cooley's anemia, target-shaped anemia) is a hereditary hemolytic anemia in children. The disease develops in connection with the violation of the synthesis of normal hemoglobin and the preservation of the synthesis of fetal (fetal) hemoglobin, the formation of degenerative forms and the accelerated destruction of red blood cells.

In the USSR, thalassemia is observed in the republics of the Transcaucasus and Central Asia.

There are two main forms of the disease: thalassemia major - homozygous variant, in which clinical signs are most pronounced, and thalassemia minor - heterozygous variant (see Heredity).

Clinically marked progressive hypochromic anemia, erythroblastemia, enlarged liver and spleen, icteric staining of the skin, osteoporosis, deformation of the facial skull, the child is physically underdeveloped.

thalassemia
The blood of a patient suffering from thalassemia. Target erythrocytes are visible.

The diagnosis is established on the basis of clinical and hematological data, as well as the detection of target-like red blood cells with a central location of hemoglobin (Fig.).

Symptomatic treatment: shown blood transfusion and red blood cell mass, removal of the spleen. The effect is temporary. The prognosis depends on the form of the disease. Patients live to mature age only with small thalassemia, occurring with less pronounced clinical manifestations. See also Anemia (in children), Hemolytic anemia, Hereditary diseases.