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Journal of Tropical Pediatrics 1990 36(1):43-45; doi:10.1093/tropej/36.1.43
© 1990 by Oxford University Press
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Beta-thalassemia/Haemoglobin E Disease in Vietnam

Nguyen Cong Khanh, Le Thi Thu, Duong Ba Truc, Dinh Phuong Hoa, Ta Thu Hoa and Tran Hong Ha

Institute for Protection of Children's Health Hanoi, Vietnam

A clinical and haematological study of 75 patients with beta-thalassemia/haemoglobin E (HbE) in Vietnam is described. The clinical picture is similar to thalassemia major. Anaemia is often severe, haemoglobin was 5.0±1.6g/dl. Splenomegaly was almost consistently detected. Haemochromatosis was clear. Both red cell indices and morphology showed hypochromicity and microcytosis, the MCH was 23.3±2.9 pg, the MCV was 81.5±11 fl; anisocytosis, poiklocytosis, tear drop cells, leptocytosis, target cells, and polychromasia were always observed. The osmotic fragility of erythrocytes was increased. The erythrocytic lifespan was shortened, about 7–15 days and the erythrocytes were destroyed in the spleen in 63 per cent of cases. Depending on whether it was beta +–thalassemia/HbE or beta °–thalassemia/HbE, HbF ranged from 22.8±7.2 to 57±12.7 per cent; HbE from 30.1±12.2 to 42.7±13 per cent; and HbA1 was decreased down to from only 46.8±13.5 to 0 per cent.


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