INTERACTION OF HEREDITARY SPHEROCYTOSIS AND ALPHA-THALASSEMIA - A FAMILY STUDY
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AbstractWe 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.
All Author(s) ListLI CK, NG MHL, CHEUNG K, LAM TK, SHING MMK
Journal nameActa Haematologica
Year1994
Month1
Day1
Volume Number91
Issue Number4
PublisherKARGER
Pages201 - 205
ISSN0001-5792
LanguagesEnglish-United Kingdom
KeywordsALPHA-THALASSEMIA; HEREDITARY SPHEROCYTOSIS; OSMOTIC RESISTANCE
Web of Science Subject CategoriesHematology; HEMATOLOGY

Last updated on 2020-03-08 at 06:19