Interaction of hereditary spherocytosis and alpha thalassaemia: a family study.
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.
Department of Paediatrics, Prince of Wales Hospital, Chinese University of Hong Kong.
C K
CK
Li
M H
MH
Ng
K L
KL
Cheung
T K
TK
Lam
M M
MM
Shing
D002648:Child; D002681:China; D004909:Erythrocyte Indices; D005260:Female; D006461:Hemolysis; D006801:Humans; D009996:Osmotic Fragility; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000150:complications*; D017085:alpha-Thalassemia / Q000097:blood / Q000150:complications*