Beta-thalassemia/haemoglobin E disease in Vietnam

J Trop Pediatr. 1990 Feb;36(1):43-5. doi: 10.1093/tropej/36.1.43.

Abstract

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.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Erythrocytes / pathology*
  • Female
  • Hemoglobin E / genetics*
  • Hemoglobinopathies / diagnosis*
  • Hemoglobinopathies / genetics
  • Hemoglobins, Abnormal / genetics*
  • Humans
  • Infant
  • Male
  • Thalassemia / diagnosis*
  • Thalassemia / genetics
  • Vietnam

Substances

  • Hemoglobins, Abnormal
  • Hemoglobin E