[The problem of sickle cell disease in Africa]

Sickle cell disease is a genetic blood disorder caused by a glutamic to valine acid substitution in the beta chain of the hemoglobin protein. It was first reported in the United States where most research has been carried out on subjects of African descent. It is diffused throughout the world. Epidemiological data show that the highest incidence of sickle cell anemia is in sub-Saharan Africa where the severest forms are often fatal in children under the age of 5 years. The clinical course of the disease in Africa is comparable to that described in industrialized countries. The three cardinal symptoms are hemolytic anemia, painful episodes, and susceptibility to infection. Genotype and phenotype variations have been observed from one zone to another in Africa. Greater severity is due to a combination of various factors including constant coexistence with Plasmodium falciparum malaria and omnipresence of pyogenic factors as well as to the unfavorable demographic setting involving endogamy, poor healthcare facilities, and poor socio-economic conditions. A hundred years of research has provided a good understanding of the pathophysiological mechanisms that can sometimes be improved by to primary hydroxyurea therapy. Sickle cell disease remains a major health problem in Africa where patients do not currently benefit from the same treatment as in industrial countries.