R. Mouele et al., alpha-thalassemia in Bantu population from Congo-Brazzaville: Its interaction with sickle cell anemia, HUMAN HERED, 50(2), 2000, pp. 118-125
Deletional alpha(+)-thalassemia (-alpha(3.7)) was investigated in four grou
ps of unrelated individuals from the Bantu population (newborns, normal adu
lts, sickle cells trait carriers, sickle cell anemia patients) of Brazzavil
le, Congo, The frequency of the (-alpha 3.7) chromosome was similar between
newborns (f = 0.40) and adult subjects (f = 0.36), and between sicklers an
d nonsickler subjects. The frequency of the (-alpha(3.7)) chromosome in sic
kle cell anemia patients (SS patients) did not change when age was stratifi
ed. The hematological characteristics of SS patients with (-alpha/alpha alp
ha, -alpha/-alpha) and without (alpha alpha/alpha alpha) alpha(+)-thalassem
ia were similar to those reported in Jamaican and US sickle cell anemia pat
ients, alpha(+)-Thalassemia had an effect on the percentage of hemoglobin S
in sickle cell trait carriers. Thus, the high frequency of alpha(+)-thalas
semia in the Congolese population presumably results from this disorder hav
ing a selective advantage favoring survival, However, the frequency of alph
a(+)-thalassemia was not affected by age, Although in this selective tropic
al environment, alpha(+)-thalassemia as elsewhere markedly affects the hema
tological characteristics of sickle cell anemia patients, however our data
provide no evidence that alpha(+)-thalassemia increases survival of SS pati
ents, Copyright (C) 1999 S. Karger AG, Basel.