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HEMOLYTIC ANEMIA SECONDARY TO A FAMILIAL (HEREDITARY) DEFECT IN HEMOGLOBIN SYNTHESISReport of a Case with Radioiron Studies
HAROLD MILLS, M.D.;
REX L. HUFF, M.D.;
MARCUS A. KRUPP, M.D.;
JOSEPH F. GARCIA, M.A.
Arch Intern Med. 1950;86(5):711-726.
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IN RECENT years there has been increased interest in familial and acquired anemias associated with erythrocytic inclusion bodies.1 Simultaneously, the hereditary transmission of certain hematological disorders has received attention.2 The purpose of this report is to present an investigation which has led us to propose that there exists a type of hemolytic anemia secondary to a hereditary defect in hemoglobin synthesis and associated with rubricytic inclusion bodies. It is our opinion that mechanisms heretofore not generally recognized have been demonstrated in this study.
BACKGROUND
A. Familial Anemias.
—The familial nature of certain anemias is well known. Familial hemolytic anemia (familial spherocytosis) is transmitted as a mendelian dominant trait, and the genetics of hereditary leptocytosis (thalassemia; Mediterranean disease) and sickle cell disease have been clarified recently. Valentine and Neel2e postulated that thalassemia major may be due to homozygosity for a factor which when
. . . [Full Text PDF of this Article]
Author Affiliations
Resident in Medicine, San Francisco Veterans Administration Hospital; Chief, Laboratory Service, San Francisco Veterans Administration Hospital; SAN FRANCISCO
From the San Francisco Veterans Administration Hospital (Drs. Mills and Krupp) and the Donner Laboratory, Division of Medical Physics, University of California, Berkeley, Calif. (Dr. Huff and Mr. Garcia).
Footnotes
Postdoctorate Fellow, Atomic Energy Commission.
This work was supported in part by the Atomic Energy Commission and in part by the Veterans Administration and is published with the approval of the Chief Medical Director. The statements and conclusions published by the authors are the result of their own studies and do not necessarily reflect the opinion or policy of the Veterans Administration.
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