[Hemoglobin C (alpha 2 beta 2 6Glu-->lys). Study of 19 heterozygote AC carriers and of 5 cases of double hemoglobinopathy SC]. / Hémoglobine C (alpha 2 beta 2 6Glu-->Lys). Etude de 19 porteurs hétérozygotes AC et de 5 cas de double hémoglobinopathie SC.

ABSTRACT

This paper describes clinical findings in subjects heterozygous for hemoglobin C and in compound heterozygotes SC. Most of our patients were coming from lands bordering the Guinea Gulf, on the inside of the loop made by the river Niger and from Haiti, which was populated by African slaves coming mainly from the Golden Coast (actually Ghana). Heterozygotes are asymptomatic and do not suffer from anemia. Age at diagnosis is higher than for SCA, although one of our cases was diagnosed at age one. Overwhelming infections or major anemia were not observed. Basal hemolysis remained moderate. HbF levels were not increased as they may be in sickle cell anemia. Splenomegaly was observed in 2/5 patients. Among severe complications we encountered, we must mention one case of foetal death, one tibial infarct, one case of pure red cell aplasia caused by parvovirus B19 infection, and one case of proliferative retinopathy.