Hemoglobin Beograd (α2β2121 Glu→Val) Interacting with β-Thalassemia

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Abstract
Hematological and biochemical findings in a family with hemoglobin (Hb) Beograd interacting with β-thalassemia are presented. Hb Beograd (α2β2121 Gul→Val) was found in 3 members. In two members it interacted with β-thalassemia. These two double heterozygotes had anemia of intermediate severity and splenomegaly. Studies with 51Cr and 59Fe showed a shortened life span of red cells and ineffective erythropoiesis. The abnormal Hb amounted to 86–87%, and Hb F to 5–7%. No Hb A was present. One subject of the family was heterozygous for Hb Beograd. He showed normal clinical and hematological findings. The abnormal hemoglobin was 38%. Four members of the family were heterozygotes for β-thalassemia. The interaction between β-thalassemia and β-chain variants is discussed.

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