Major sickle cell syndromes: epidemiological, clinical and outcome data

Abstract

Background: Major sickle cell syndromes is a group of inherited diseases that includes sickle cell anemia, Hb SC and Hb SBetathalassemia. The aim of our study was to determine the epidemiological, clinical, hematological profiles and outcome of children with major sickle disease syndromes in a Hospital in south Tunisia. This is a retrospective study including children aged between 5 months and 14 years with SS sickle cell anemia or S β thalassemia. We collected 18 cases (13 boys and 5 girls). The average age at diagnosis was 30 months. During follow up, 9 patients developed vaso-occlusive crises, two patients developed brain stroke and four patients had acute chest syndrome. Conclusion: The diagnosis and management of SS sickle-cell disease are relatively late because of the absence of neonatal screening and low socioeconomic level of families.