Coinheritance of sickle cell anemia and hereditary spherocytosis
PEDIATRIC BLOOD & CANCER, vol.51, no.4, pp.560-563, 2008 (SCI-Expanded, Scopus)
- Publication Type: Article / Article
- Volume: 51 Issue: 4
- Publication Date: 2008
- Doi Number: 10.1002/pbc.21642
- Journal Name: PEDIATRIC BLOOD & CANCER
- Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Page Numbers: pp.560-563
- Istanbul University Affiliated: Yes
Abstract
To date only three siblings with coinheritance of sickle cell anemia (SCA) and hereditary spherocytosis (HS) have been reported. We here describe a 17-year-old boy who experienced episodes of hemolysis and had a large spleen. The diagnosis of SCA was confirmed by hemoglobin electrophoresis (HbS 88.9%) and genetic analysis (homozygote HbSS mutation). The diagnosis of HS was established by an osmotic fragility test, performed twice. A splenectomy was performed, and following Surgery the hemoglobin concentration was maintained between 9 and 11 g/dl without further transfusion requirements. This patient was the fourth reported case with co-existence of two different genetically transmitted hemolytic anemias.