Volume 1

The haemolytic anaemias, congenital and acquired / by J.V. Dacie.

  • John Vivian Dacie
Date:
1960-1967
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: The haemolytic anaemias, congenital and acquired / by J.V. Dacie. Source: Wellcome Collection.

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    provides strong support for the concept that Hb-A, Hb-S and Hb-C are alleles. Further extensions of this work have recently been reported. Benzer, Ingram and Lehmann (1958) have differentiated three different types of Hb-D, all with chemical changes affecting different parts of the polypeptide chains of the haemoglobin half molecule, and Ingram (1959) reports that in Hb-E lysine has been substituted for glutamic acid, as in Hb-C, but this time in a different part of the molecule. The finding that “Hb-D” consists of at least three haemoglobins which have different chemical constitutions, although having identical electrophoretic, chromatographic and solubility properties, is remarkable. It suggests that haemoglobins with apparently similar physico-chemical properties, discovered in widely separated parts of the world, may also not be identical when subjected to critical chemical analysis (see Vella, Ager and Lehmann, 1958). The results of further studies can hardly fail to be of great interest. Erythrocyte Survival Studies in Sickle-Cell Disease and Allied Disorders Measurements of cell survival have demonstrated, as might be anticipated from clinical data, that the life-span of the erythrocytes is considerably diminished in Hb-S disease and diminished to a minor degree in Hb-S/Hb-C disease, etc. Normal erythrocytes transfused to patients with hsemoglobinopathies characteristically survive normally. The first direct measurements of the life-span in vivo of the erythrocytes in Hb-S disease were reported by Singer, Robin, King and Jefferson (1948) and Callender, Nickel, Moore and Powell (1949). Using the Ashby technique, marked differences in the survival of the transfused cells were observed; a proportion of the cells disappeared rapidly from the circulation, others were eliminated far more slowly. Singer and Fisher (1952) correlated the difference in survival time with the relative amounts of Hb-S and Hb-F present and thought that the cells containing the greatest amount of Hb-F survived the longest. Weinstein and co-workers (1954) studied the survival of the erythrocytes of a small number of patients in their own circulations, using 51 Cr. As expected, a major increase in cell destruction was found in Hb-S disease (one patient gave a definitely two-component curve of elimination) and minor increases were found in Hb-C disease and Hb-S/ Hb-C disease. The results were normal in Hb-S trait and Hb-C trait. James and Abbott (1955) reported the result of simultaneous [ 15 N]hsemin and [ 15 N]stercobilin studies in a patient with severe Hb-S disease. The erythrocyte half-life was computed to be 11 days, with a mean life-span