Alpha-Beta Thalassemia Disease in a Negro Family
- 28 July 1966
- journal article
- case report
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 275 (4) , 176-181
- https://doi.org/10.1056/nejm196607282750402
Abstract
CONVINCING evidence has been presented that the term thalassemia does not describe a specific or homogeneous entity. Bather, it refers to a group of heritable defects of hemoglobin synthesis manifested by hypochromic microcytic anemia. The most clearly defined of these have been designated respectively as α thalassemia and β thalassemia because the genetically determined blocks of hemoglobin synthesis primarily affect these polypeptide subunits.1 β-thalassemia in the heterozygous state is characterized by significant elevations of hemoglobin A2 in more than 90 per cent of cases.2 , 3 This form of thalassemia "interacts" with abnormal β-chain hemoglobin genes to produce a hematologic disease . . .Keywords
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