부산대학교 도서관

A case of hereditary stomatocytosis is reported. A 43-year-old female with persistent anemia and jaundice was admitted to the Hospital of Jinchi Medical School. She was born uneventfully and did not have a severe neonatal jaundice. She had been noticed to have anemia and jaundice since she was 16 years old. However, she had been well except for an increase of these symptoms in occasions of overwork or upper respiratory infection.On physical examination, she had anemia, Jaundice and hepatosplenomegaly. Laboratory data revealed: stomatocytes 30∼40% on peripheral blood film, RBC 264×104/mm3, Hb 9.2 g/dl, Ht 31.0%, reticulocyte count 19.8%, serum total bilirubin 1.9 mg/dl (indirect bilirubin 1.1 mg/dl), apparent half life of the 51Cr-labeled red cells 5.8 days, negative Coombs' test, increased osmotic fragility of red cells, negative Ham's test, erythroid hyperplasia in bone marrow, normal enzyme activities in erythrocytes, normal electrophoretic pattern of hemoglobin.Family studies showed that her father and aunt had the same abnormalities as those of the patient.