Hematological observations on Arabian SS patients with a homozygosity or heterozygosity for a beta S chromosome with haplotype #31.
Hematological and hemoglobin composition data are presented for seven Arabian SS patients with mild disease and with high Hb F levels varying between 21 and 34%. Four patients were homozygous for a beta S chromosome with a specific haplotype (#31). The data for these four patients were similar to those for three other SS patients (and for five patients reported earlier, Ref. 2) who were heterozygous for the same beta S chromosome (#31) and for a beta S chromosome with another haplotype (mainly #19). These data offer additional evidence indicating that the increased gamma chain production is specific for the beta S chromosome with haplotype #31. The similarities in hematological data and Hb F levels between these two groups of SS patients and the normal Hb F value in Hb S heterozygotes with beta S chromosome (#31) support the suggestion that the increased Hb F production mainly occurs in response to the anemia of the sickle cell disease.
Kutlar
A
A
Hattori
Y
Y
Bakioglu
I
I
Kutlar
F
F
Kamel
K
K
Huisman
T H
TH
D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002877:Chromosomes, Human / Q000032:analysis*; D004247:DNA / Q000032:analysis; D005260:Female; D005319:Fetal Hemoglobin / Q000235:genetics; D005914:Globins / Q000235:genetics*; D006238:Haploidy; D006451:Hemoglobin, Sickle / Q000235:genetics*; D006579:Heterozygote; D006720:Homozygote; D006801:Humans; D008297:Male; D010375:Pedigree; D012529:Saudi Arabia
D006451:Hemoglobin, Sickle; D005914:Globins; D004247:DNA; D005319:Fetal Hemoglobin