Hemoglobin E in Indochinese refugees

Abstract

Hemoglobin E, a beta-chain variant commonly found in Southeast Asian populations, is relatively unfamiliar to American physicians. The hematologic features of 55 patients with hemoglobin E (8 homozygote, 46 heterozygote and 1 genotype E/beta(thal)) are described and compared with previous series. The patients were immigrants from Cambodia, Laos, Thailand and Vietnam. These studies support the association of this beta-globin variant with microcythemia and suggest that it mimics the "thalassemia picture" of microcythemia out of proportion to the degree of anemia. Splenomegaly was not a characteristic. Oxidative hemolysis may occur under conditions that deplete reduced glutathione. The simultaneous presence of the beta-thalassemia trait appears to significantly worsen the prognosis.