Sickle Cell Anemia


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Related to Sickle Cell Anemia: sickle cell trait, thalassemia, Sickle cell crisis

Sickle Cell Anemia

 

one of the forms of hereditary hemolytic anemia. The distinguishing characteristic of sickle cell anemia is the sickle shape of erythrocytes during hypoxia. The disease was described in 1910 by the American physician J. B. Herrick.

Sickle cell anemia is caused by the pathological hemoglobin S, in which the amino acid valine is substituted for glutamic acid in one portion of the protein chain. The discovery of this fact in 1949 by L. Pauling and his co-workers laid the basis for the study of human molecular diseases. Sickle cell anemia occurs in Equatorial Africa; it has not been observed among the indigenous populations of America, Australia, and northern Europe.

During sickle cell anemia, erythrocytes have an increased resistance to invasion by malarial plasmodia. Homozygotic children suffer from severe forms of the disease, including hemolytic crises with jaundice, fever, progressive anemia, and abdominal pains, and usually die during early childhood or during adolescence. Heterozygotic children are usually only potential carriers of sickle-shaped erythrocytes and do not suffer from the disease. However, under hypoxia-producing conditions, for example, a jet flight, they may suffer from hemolytic crises, thromboses of cerebral vessels, and infarcts of internal organs.

Sickle cell anemia is treated with aspirin and anticoagulants in cases of crises and with transfusion in cases of severe anemia. The preventive treatment of crises in heterozygotic individuals is important.

A. I. VOROBEV

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