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AE Kulozik, BC Kar, GR Serjeant, BE Serjeant and DJ Weatherall
Nuffield Department of Clinical Medicine, University of Oxford, John
Radcliffe Hospital, England.
The alpha globin genotype of a total of 282 Indians from Orissa state has
been analyzed. The overall alpha thalassemia gene frequency is 0.29, most
frequently caused by the -alpha 3.7 and -alpha 4.2 deletions. In one family
a novel -alpha 3.5 deletion removing the alpha 1 globin gene with some of
its flanking sequences has been found, suggesting further sequence homology
of the alpha globin gene cluster 3' to the alpha 1 globin gene. Patients
with sickle cell disease and alpha thalassemia had higher hemoglobin (Hb)
levels, RBC counts, and Hb A2 levels, and lower reticulocyte counts, MCV,
MCH, and Hb F levels than those with a normal alpha genotype. The frequency
of splenomegaly was not influenced by the alpha globin genotype. A higher
prevalence of alpha thalassemia was found in patients greater than or equal
to 10 years of age than in the younger group, suggesting a possible
advantageous effect of alpha thalassemia on the survival of patients with
sickle cell disease.
This article has been cited by other articles:
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| Copyright © 1988 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||