Beta-thalassemia/haemoglobin E disease in 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. Anemia is often severe, haemoglobin was 5.0 +/- 1.6 g/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(0)-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.
Nguyen
C K
CK
Institute for Protection of Children's Health, Hanoi, Vietnam.
Le
T T
TT
Duong
B T
BT
Dinh
P H
PH
Ta
T H
TH
Tran
H H
HH
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004912:Erythrocytes / Q000473:pathology*; D005260:Female; D006446:Hemoglobin E / Q000235:genetics*; D006453:Hemoglobinopathies / Q000175:diagnosis* / Q000235:genetics; D006455:Hemoglobins, Abnormal / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D013789:Thalassemia / Q000175:diagnosis* / Q000235:genetics; D014744:Vietnam
D006455:Hemoglobins, Abnormal; D006446:Hemoglobin E