TY - JOUR
T1 - Alpha-thalassemia is related to prolonged survival in sickle cell anemia
AU - Mears, J. G.
AU - Lachman, H. M.
AU - Labie, D.
AU - Nagel, R. L.
PY - 1983
Y1 - 1983
N2 - We have determined the frequency of deletional α-thalassemia in black populations in the USA and Africa that harbor sickle cell anemia. In normals, the frequency of the chromosome bearing a deletion of one of the two normal α gene loci, designated (-α), ranged from 0.12 to 0.16, and in sickle trait subjects, the frequency ranged from 0.18 to 0.20. By contrast, in sickle cell anemia subjects, the frequency was significantly greater and ranged from 0.22 to 0.33. Analysis demonstrated that the greater frequency in the last group was primarily a result of an increased number of subjects with α-thalassemia trait (also called homozygous α-thalassemia-2). In addition, the frequency of the (-α) chromosome was found to increase progressively with age, supporting the hypothesis that 1/2 a-thalassemia is favorable to the survival of subjects with sickle cell anemia. Thus, individuals who inherit α-thalassemia and sickle cell anemia may represent a subgroup of patients with a longer life expectancy.
AB - We have determined the frequency of deletional α-thalassemia in black populations in the USA and Africa that harbor sickle cell anemia. In normals, the frequency of the chromosome bearing a deletion of one of the two normal α gene loci, designated (-α), ranged from 0.12 to 0.16, and in sickle trait subjects, the frequency ranged from 0.18 to 0.20. By contrast, in sickle cell anemia subjects, the frequency was significantly greater and ranged from 0.22 to 0.33. Analysis demonstrated that the greater frequency in the last group was primarily a result of an increased number of subjects with α-thalassemia trait (also called homozygous α-thalassemia-2). In addition, the frequency of the (-α) chromosome was found to increase progressively with age, supporting the hypothesis that 1/2 a-thalassemia is favorable to the survival of subjects with sickle cell anemia. Thus, individuals who inherit α-thalassemia and sickle cell anemia may represent a subgroup of patients with a longer life expectancy.
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U2 - 10.1182/blood.v62.2.286.bloodjournal622286
DO - 10.1182/blood.v62.2.286.bloodjournal622286
M3 - Article
C2 - 6307429
AN - SCOPUS:0020563256
SN - 0006-4971
VL - 62
SP - 286
EP - 290
JO - Blood
JF - Blood
IS - 2
ER -