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

Abstract

Despite the unicity of its genetic mutation, Sickle cell homozygosity presents different clinical features. Our objectives were to evaluate disease severity in Senegalese patients. Sixty (60) homozygous sickle cell patients were followed up monthly during one year and disease severity was assessed using the "severity index" (SI) which is resulting from epidemiologic, clinic 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 complications linked to anemia or ischemia. Mean hemoglobin value was 8.1 g/dl and mean Hb F was 8.2%. Low seric ferritin was found in 1.7% of patients. Benign form of homozygous sickle cell anemia (SI< or =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 certainly because some severe cases were identified in our patients. The identification of all these factors might contribute to a better follow up of sickle cell disease.

Clinical and molecular characteristics of sickle cell anemia in the northeast of Brazil

“…However, since there was only a small number of CAR/CAR haplotypes the associations involving the CAR/CAR genotype with hematological or clinical findings seen in our study and discussed above should be interpreted with caution because some of these associations could be due to chance sampling events. It is interesting to note that we found that the outpatient with the Sen/Atypical haplotype presented low HbF levels and had no history of erythrocyte therapy, possibly suggesting that the Senegal haplotype is associated with mild clinical manifestations, as previously reported by Diop et al (1999).…”

Clinical Profile of Sickle Cell Anaemia in Adolescent and Adult Patients in Central India

“…In present study mean HbF was 21.91 ± 7.27%, being slightly higher in females as compared to males. These values are consistent with the findings observed by Shrikande AV et al 9 HbF production was genetically determined and Arab-Indian haplotype is associated with higher HbF levels as compared to Cameroon haplotype. This explains the low value of HbF in Nigerian population by Kotila TR et al 4 S Diop et al 10 reported mean age of onset of first symptom in sickle cell anaemia was 9.8 years in Senegal.…”

Profil biochimique et hématologique des patients drépanocytaires homozygotes en phase stationnaire au centre National de Transfusion Sanguine de Dakar

“…Le taux moyen de l'hémoglobine F était de 9,5 ± 8,3%. Des études menées au Sénégal ont montré chez les patients drépanocytaires homozygotes, un taux moyen d'hémoglobine F de 6,8% chez les enfants (Diagne et al, 2000) et de 8,2% chez les adultes (Diop et al, 1999). L'hémoglobine F constitue le principal constituant hémoglobinique durant la période prénatale.…”