[Clinical and hematological profile of Lepore Hemoglobin in Ivory Coast].
Out of 97320 hemoglobin electrophoreses performed in Abidjan between January 1976 and January 1991, all subjects with hemoglobin Lepore were isolated. This trait was identified by three techniques, i.e., alkaline pH electrophoresis, acid pH electrophoresis, and isoelectric focusing. Seventy-nine cases of hemoglobin Lepore were observed. All were heterozygotes with type HbA-Lepore (n = 54), HbC-Lepore (n = 8) or HbS Lepore (n = 17). Where heterozygosis A and C had clinically silent, heterozygosis Hb-S Lepore resulted in a moderate chronic hemolytic anemia and, in all cases, painful episodes similar to those observed during homozygote sickle-cell disease. However the onset of episodes was later and their occurrence was less frequent. On hemograms, the Lepore trait (HbA Lepore) appeared as a pseudo-polyglobulia with microcytosis; similar features were observed for heterozygosis HbC Lepore. Heterozygosis HbS Lepore caused moderate anemia (mean hemoglobin level: 10.66 g/dl) and microcytosis (MGV = 68.8 fl). The characteristics show that the clinical and hematological behavior of hemoglobin Lepore, a rare hemoglobin, is similar to heterozygous beta-thalassemia.
Sangare
A
A
Service d'Hématologie, Centre Hospitalier Universitaire de Yopougon, Faculté de Médecine, Abidjan, Côte-d'Ivoire.
Sanogo
I
I
Meite
M
M
Segbena
A
A
Toure
A H
AH
Elenga
J P
JP
Siransy
L
L
Allangba
O
O
D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000209:etiology; D001797:Blood Protein Electrophoresis / Q000379:methods; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D007560:Cote d'Ivoire; D003434:Crossing Over, Genetic; D005260:Female; D006580:Genetic Carrier Screening; D006441:Hemoglobin A / Q000032:analysis* / Q000235:genetics; D006444:Hemoglobin C / Q000032:analysis* / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000032:analysis* / Q000235:genetics; D006453:Hemoglobinopathies / Q000097:blood* / Q000150:complications / Q000453:epidemiology* / Q000235:genetics; D006455:Hemoglobins, Abnormal / Q000032:analysis* / Q000235:genetics; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000209:etiology; D010641:Phenotype; D011159:Population Surveillance
D006451:Hemoglobin, Sickle; D006455:Hemoglobins, Abnormal; D006444:Hemoglobin C; D006441:Hemoglobin A; C037659:hemoglobin Lepore
Profil clinique et hématologique de l'hémoglobine Lepore en Côte d'Ivoire.