SICKLE-CELL DISORDER, BETA-GLOBIN GENE-CLUSTER HAPLOTYPES AND ALPHA-THALASSEMIA IN NEONATES AND ADULTS FROM GUADELOUPE

We have studied haplotype of beta(S) chromosome and alpha-globin gene status in 534 patients (255 adults and 279 children of whom 159 neonat es) from Guadeloupe with various sickle cell-related conditions, namel y SS (n = 298), SC (n = 170), S-beta-thal (n = 56), and other rare for ms (n = 10). Haplotype data on PS chromosomes confirm our previous obs ervation that Benin type is the most prevalent (75%) beta(S) chromosom e in Guadeloupe, in disagreement with the historical records. Comparis on of the frequency of distribution of various beta(S) haplotypes betw een neonates and adults on the one hand and between SS and SC cases on the other shows that the current beta(S) haplotype distribution in th is island is not distorted by haplotype-related differential survival. We also show that the frequency of alpha-thalassemia (-3.7 kb) in Gua deloupe is one of the highest recorded in this region involved in Atla ntic slave trade and also failed to reveal any age dependent increase in frequency. We conclude that the African component of Guadeloupe is distinct from that of Brazil and Cuba but is close to that of Jamaica. (C) 1997 Wiley-Liss, Inc.