Alpha-thalassemia is related to prolonged survival in sickle cell anemia

J. G. Mears, Herbert M. Lachman, D. Labie, R. L. Nagel

Research output: Contribution to journalArticle

53 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)286-290
Number of pages5
JournalBlood
Volume62
Issue number2
StatePublished - 1983
Externally publishedYes

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alpha-Thalassemia
Thalassemia
Sickle Cell Anemia
Chromosomes
Bearings (structural)
Survival
Ports and harbors
Genes
Life Expectancy
Population

ASJC Scopus subject areas

  • Hematology

Cite this

Mears, J. G., Lachman, H. M., Labie, D., & Nagel, R. L. (1983). Alpha-thalassemia is related to prolonged survival in sickle cell anemia. Blood, 62(2), 286-290.

Alpha-thalassemia is related to prolonged survival in sickle cell anemia. / Mears, J. G.; Lachman, Herbert M.; Labie, D.; Nagel, R. L.

In: Blood, Vol. 62, No. 2, 1983, p. 286-290.

Research output: Contribution to journalArticle

Mears, JG, Lachman, HM, Labie, D & Nagel, RL 1983, 'Alpha-thalassemia is related to prolonged survival in sickle cell anemia', Blood, vol. 62, no. 2, pp. 286-290.
Mears, J. G. ; Lachman, Herbert M. ; Labie, D. ; Nagel, R. L. / Alpha-thalassemia is related to prolonged survival in sickle cell anemia. In: Blood. 1983 ; Vol. 62, No. 2. pp. 286-290.
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