Popis: |
— Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity of clinical features, ranging from an asymptomatic condition to a fulminate haemolytic anaemia. Although a positive family history of spherocytosis increases the risk for this disorder, it may be sporadic in some cases. Seventeen years old girl presented with mild anaemia, jaundice and moderate splenomegaly. Her haematological parameters supported diagnosis of hereditary spherocytosis. Appropriate treatment was started with an advice to patient to undergo splenectomy as an active part of management of the disorder. Mother and other siblings were normal. |