Hemoglobin E: a common hemoglobinopathy among children of Southeast Asian origin.
With the recent immigration of Southeast Asians to Canada, hemoglobin E has become a frequent diagnosis. The clinical and hematologic findings in 42 children (mean age 4.3 years) with hemoglobin E are presented. There were 33 heterozygotes (having hemoglobin E trait), 6 homozygotes (having hemoglobin EE) and 3 double heterozygotes (having hemoglobin E-beta-thalassemia). The heterozygotes had low-normal hemoglobin levels and mean corpuscular volumes; coexisting iron deficiency, present in 62% of these children, resulted in substantially lower hemoglobin levels, very low mean corpuscular volumes and lower than expected levels of hemoglobin E on electrophoresis. The children with hemoglobin EE were only slightly anemic, but those with hemoglobin E-beta-thalassemia had severe anemia and required long-term transfusion therapy. Nutritional factors and parasitic infestations were the main causes of iron depletion, which was common, particularly in children less than 2 years old (87%). Physicians of patients of Southeast Asian origin should be aware of the clinical and hematologic presentation of these hemoglobinopathies.
D000293:Adolescent; D000747:Anemia, Hypochromic / Q000097:blood; D001210:Asia, Southeastern / Q000208:ethnology; D002170:Canada; D002675:Child, Preschool; D005260:Female; D006446:Hemoglobin E / Q000032:analysis* / Q000235:genetics; D006453:Hemoglobinopathies / Q000453:epidemiology*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006579:Heterozygote; D006801:Humans; D008297:Male; D009752:Nutritional Status; D010272:Parasitic Diseases / Q000150:complications; D013789:Thalassemia / Q000097:blood
D006455:Hemoglobins, Abnormal; D006446:Hemoglobin E