H. Wajcman et al., HbF in the adult: could its composition discriminate normal from abnormal foetal globin gene expression?, CR AC S III, 323(11), 2000, pp. 975-981
Citations number
15
Categorie Soggetti
Multidisciplinary,"Experimental Biology
Journal title
COMPTES RENDUS DE L ACADEMIE DES SCIENCES SERIE III-SCIENCES DE LA VIE-LIFE SCIENCES
The relative proportions of the two gamma chain species ((G)gamma and (A)ga
mma) have been reinvestigated in newborns, during the physiological switch
from foetal to adult haemoglobin, and in adults with some persistent expres
sion of HbF. In newborns, with about 80 % HbF, the (G)gamma percentage was
close to 70 % while in adult RBC, with less than 0.5 % HbF, the (G)gamma ch
ain was almost non-detectable and may reflect the completion of the foetal
to adult switch. Conversely, in adult patients with HbF above 0.6 %, usuall
y accompanying some degree of marrow stimulation, the relative ratio of (G)
gamma varied between 40 and 60 %, independently of HbF level. This ratio co
rresponds to what has been described in the literature as being the adult t
ype of HbF. In all the cases where we found higher levels of (G)gamma, the
results could be explained by the presence of a specific genetic background
such as the Senegalese haplotype in sickle cell disease. (C) 2000 Academie
des sciences/ Editions scientifiques et medicales Elsevier SAS.