RT Journal Article
SR Electronic
T1 Hemoglobin E: a common hemoglobinopathy among children of Southeast Asian origin
JF Canadian Medical Association Journal
JO CMAJ
FD Canadian Medical Association
SP 39
OP 42
VO 137
IS 1
A1 Katsanis, E.
A1 Luke, K. H.
A1 Hsu, E.
A1 Yates, J. R.
YR 1987
UL http://www.cmaj.ca/content/137/1/39.abstract
AB 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.