TY - JOUR
T1 - -Globin gene haplotype in Hb SC disease
AU - Steinberg, Martin H.
AU - Nagel, Ronald L.
AU - Lawrence, Christine
AU - Swaminathan, Venkataramani
AU - Lu, Zhi Hong
AU - Plonczynski, Maria
AU - Harrell, Audrey
PY - 1996
Y1 - 1996
N2 - We asked the question, is the haplotype found with the sickle hemoglobin gene associated with different hematological characteristics in patients who were combined heterozygotes for sickle hemoglobin and hemoglobin C (Hb SC disease)? In 73 adults with Hb SC disease, a Benin haplotype chromosome was present in 56%, and Bantu (or Central African Republic; CAR), Senegal, and atypical haplotype chromosomes were found in 25%, 6%, and 12%, respectively. No significant differences were found in hematological characteristics or fetal hemoglobin levels of patients with Benin/C, CAR/C, Senegal/C, and atypical/C haplotypes. There were 71% C I, 18% C II, and 11% other β(c) haplotypes. Fetal hemoglobin levels are lower in Hb SC disease than in sickle-cell anemia. Perhaps because haplotype has no discernible effect on fetal hemoglobin level in Hb SC disease, it does not modulate its hematological features.
AB - We asked the question, is the haplotype found with the sickle hemoglobin gene associated with different hematological characteristics in patients who were combined heterozygotes for sickle hemoglobin and hemoglobin C (Hb SC disease)? In 73 adults with Hb SC disease, a Benin haplotype chromosome was present in 56%, and Bantu (or Central African Republic; CAR), Senegal, and atypical haplotype chromosomes were found in 25%, 6%, and 12%, respectively. No significant differences were found in hematological characteristics or fetal hemoglobin levels of patients with Benin/C, CAR/C, Senegal/C, and atypical/C haplotypes. There were 71% C I, 18% C II, and 11% other β(c) haplotypes. Fetal hemoglobin levels are lower in Hb SC disease than in sickle-cell anemia. Perhaps because haplotype has no discernible effect on fetal hemoglobin level in Hb SC disease, it does not modulate its hematological features.
KW - Hemoglobinopathy
KW - Sickle hemoglobin
KW - Sickle-cell disease
UR - http://www.scopus.com/inward/record.url?scp=0029666051&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1096-8652(199607)52:3<189::AID-AJH9>3.0.CO;2-P
DO - 10.1002/(SICI)1096-8652(199607)52:3<189::AID-AJH9>3.0.CO;2-P
M3 - Article
C2 - 8756085
AN - SCOPUS:0029666051
SN - 0361-8609
VL - 52
SP - 189
EP - 191
JO - American journal of hematology
JF - American journal of hematology
IS - 3
ER -