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.
Li
C K
CK
Department of Paediatrics, Prince of Wales Hospital, Chinese University of Hong Kong.
Ng
M H
MH
Cheung
K L
KL
Lam
T K
TK
Shing
M M
MM
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*