Hemoglobin E in Indochinese refugees.
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.
D000328:Adult; D001210:Asia, Southeastern / Q000208:ethnology; D002675:Child, Preschool; D005260:Female; D006446:Hemoglobin E / Q000235:genetics*; D006455:Hemoglobins, Abnormal / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012036:Refugees; D013789:Thalassemia / Q000235:genetics*; D014481:United States
D006455:Hemoglobins, Abnormal; D006446:Hemoglobin E