Interaction of hereditary spherocytosis and alpha thalassaemia: a family study

Acta Haematol. 1994;91(4):201-5. doi: 10.1159/000204335.

Abstract

We report here a family of patients with coexistent hereditary spherocytosis and alpha-thalassaemia. The different clinical presentations of the family members were affected by the severity of the alpha-thalassaemia. The haemolytic effect of hereditary spherocytosis was modulated by the increased osmotic resistance of thalassaemia in the patients with both disorders. Coexistence of haemoglobin H disease and hereditary spherocytosis resulted in an asymptomatic state. In contrast, coexistence of alpha-thalassaemia trait and hereditary spherocytosis was still haemolytic. The different shapes of the osmotic curves in the family members correlated with the severity of their symptoms.

Publication types

  • Case Reports

MeSH terms

  • Child
  • China
  • Erythrocyte Indices
  • Female
  • Hemolysis
  • Humans
  • Osmotic Fragility
  • Spherocytosis, Hereditary / blood
  • Spherocytosis, Hereditary / complications*
  • alpha-Thalassemia / blood
  • alpha-Thalassemia / complications*