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  • 1
    Publication Date: 1965-01-01
    Description: Two adult patients with sickle cell anemia of blood group A and A2B respectively, received sufficient transfusions of group O blood to maintain nearly normal hemoglobin concentrations for 4 months or longer. Serial samples of the erythrocytes of each recipient were obtained by agglutination with anti-A (and anti-B) serum. The proportion of Hb F in the agglutinated erythrocytes was determined. Early in the transfusion period, a marked rise in the proportion of Hb F was noted. This rise was attributed to prolonged survival of erythrocytes which contained larger proportions of Hb F. In the later part of the transfusion period, the proportion of fetal hemoglobin declined to pre-transfusion levels or below. However, significant amounts of fetal hemoglobin in the erythrocytes of each patient were demonstrated throughout the period of study, and Fe59 incorporation into Hb F in vivo was demonstrated in one patient after 4 months of transfusion therapy. Under the conditions of these studies, synthesis of Hb F continued despite prolonged correction of the anemia. A decline in the proportion of Hb F in the erythrocytes of one patient after 5 months of transfusions suggested that Hb F synthesis may ultimately be depressed by transfusions. It was suggested that the proportion of fetal hemoglobin observed in the erythrocytes might in certain diseases reflect the degree of anemia present many months before.
    Print ISSN: 0006-4971
    Electronic ISSN: 1528-0020
    Topics: Biology , Medicine
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  • 2
    Publication Date: 1968-09-01
    Description: A new unstable hemoglobin variant, hemoglobin Gun Hill, is reported. Two members of a Caucasian family were found to be heterozygous for the abnormal hemoglobin. Evidence of mild chronic hemolysis was found in both individuals. Heinz-Ehrlich bodies were absent from the peripheral blood of the patients with hemoglobin Gun Hill, but Heinz-like inclusions were readily produced in their red cells upon in vitro incubation with the redox dyes brilliant cresyl blue or new methylene blue. Precipitation of hemoglobin occurred when solutions of hemoglobin Gun Hill were exposed to the dyes or were heated to 50 C. Previous studies have shown that there is a deletion of five amino acids in the β chains of hemoglobin Gun Hill. This results in impaired heme binding, and the abnormal molecule lacks half the expected number of heme groups. These structural alterations are probably responsible for the instability of the abnormal hemoglobin. Studies of the relative rates of synthesis of hemoglobins A and Gun Hill, using in vitro and in vivo technics, indicated an increased turnover rate for the variant protein. The evidence also suggested that hemoglobin Gun Hill is synthesized more rapidly than hemoglobin A.
    Print ISSN: 0006-4971
    Electronic ISSN: 1528-0020
    Topics: Biology , Medicine
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  • 3
    Publication Date: 1969-08-01
    Description: A Jewish family in which Hb L Ferrara (α247 Asp → Gly β2) occurred is reported. Studies of some of the properties of this hemoglobin demonstrated that its oxygen equilibria, number of readily reactive-SH groups, and spectro-photometric tyrosine titration were indistinguishable from Hb A. Nevertheless, Hb LF was more unstable than Hb A at 55 C. The propositus had accelerated blood destruction although six other heterozygotes for Hb LF did not. A second defect in red cell enzymes or red cell lipids of the propositus was not demonstrable with the technics used but the possibility that the simultaneous occurrence of Hb LF and an otherwise "silent" red cell defect may lead to a hemolytic state remains an attractive explanation. The data provided by this family study did not permit a definite conclusion about the relationship of clinically evident hemolysis in the propositus to the presence of the abnormal hemoglobin.
    Print ISSN: 0006-4971
    Electronic ISSN: 1528-0020
    Topics: Biology , Medicine
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