New results in clinical severity of homozygous sickle cell anemia in Dakar, Senegal

Despite the unicity of its genetic mutation, Sickle cell homozygosity prese nts different clinical features. Our objectives were to evaluate disease se verity in Senegalese patients. Sixty (60) homozygous sickle cell patients w ere followed up monthly during one year and disease severity was assessed u sing the "severity index" (SI) which is resulting from epidemiologic, clini c and biological data. Mean age was 20.13, sex ratio was 0.87 and mean age of diagnosis was 9.8 years. 90% of patients presented vaso-occlusive crisis (2.53 per patient), 73.3% had infectious episodes (1.9 per patient), 69.3% had never been transfused and 25% of patients had presented chronic compli cations linked to anemia or ischemia. Mean hemoglobin value was 8.1 g/dl an d mean Hb F was 8.2%. Low seric ferritin was found in 1.7% of patients. Ben ign form of homozygous sickle cell anemia (SI less than or equal to 6) was found in 48.3% of patients. Our data confirm the relative good tolerance of homozygous sickle cell disease in Senegal. The haplotype Senegal may play an important role but others host and environmental factors operate certain ly because some severe cases were identified in our patients. The identific ation of all these factors might contribute to a better follow up of sickle cell disease.