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Hemoglobin. 1988;12(5-6):509-17.

The interaction of alpha-thalassemia with sickle cell anemia.

Author information

1
Hematology Service, San Francisco General Hospital, University of San Francisco, CA 94110.

Abstract

The coinheritance of alpha-thalassemia (alpha-thal) and sickle cell anemia provides a most interesting example of how separate globin gene mutations influence clinical manifestations of abnormal gene expression. Early reports in the literature contained conflicting opinions as to whether alpha-thal ameliorated the clinical consequences of sickle cell disease. With the discovery that the concentration of sickle hemoglobin (Hb S) had a profound influence on both the kinetics and extent of deoxy Hb S polymerization, it was predicted that the lower intraerythrocytic concentration of Hb S associated with alpha-thal would mitigate the clinical severity of sickle cell anemia. Moreover, the use of alpha-globin gene mapping for objectively diagnosing alpha-thal revealed that one in three Black Americans were silent carriers of alpha-thal. Thus, it followed that a great many sickle cell patients may be affected by this potentially modifying influence.

PMID:
3209393
DOI:
10.3109/03630268808991639
[Indexed for MEDLINE]

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