[Adults with sickle cell anemia in Senegal. Clinical study of 40 homozygote subjects]. / L'adulte drépanocytaire au Sénégal. Etude clinique de 40 sujets homozygotes.

ABSTRACT

While the well-documented life expectancy of patients with homozygous sickle cell anemia (SS) is 40 years in industrialized countries, this question remains unanswered in black Africa. The purpose of this prospective study was to establish the clinical phenotype for Senegal. A severity score based on 12 clinical, laboratory, radiological, and prognostic findings was calculated and correlated with age and hemoglobin F level. A total of 40 SS homozygotes over 15 years of age (mean age 25 years) were hospitalized between January 1996 and January 1998 at the Principal Hospital in Dakar. The most common events requiring hospitalization were vasoocclusive phenomena (n = 26) but the incidence of these complications declined significantly with age (p < 0.05). The mean hemoglobin level was 4.4 mmol/l and the mean hemoglobin F level was 6.2 p. 100. The incidence of visceral involvement was low (lithiasic vesicules in 17 cases, necrosis of the head of the femur in 7, and abnormal cardiac ultrasound findings in 10). Only one patient died during the study. No correlation was found between severity score and either age or hemoglobin F level. These findings confirm that the phenotype is less severe in Senegal. However they also show that organ damage is common by the time that patients reach adulthood and thus underline the need for prevention and education to improve survival of SS homozygotes in Senegal.