Volume 1

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

  • Dacie, John V. (John Vivian), Sir.
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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    or intravenously, is useless and may be dangerous—indeed, the serum-iron concentration is usually raised and the storage organs of the body also contain an excess of iron. All the vitamin preparations that have been tried seem to be valueless. 1 It is possible that cobalt may be of slight value. Berk, Burchenal and Castle (1949) reported rather doubtful improvement in one patient and other instances of possible benefit have been referred to by Wiessbecker (1951), Muratore (1951) and Heilmeyer, Muller and Schubothe (1951). Virdis (1952), on the other hand, did not observe any improvement in six children given 20 mg. of cobaltous chloride orally for 10-20 days. Its use can hardly be recommended with any enthusiasm. Blood Transfusion. This has no fundamental effect on the course of the disease. However, normal erythrocytes survive normally in uncomplicated Mediterranean anaemia, and for this reason great temporary benefit can be expected to result from transfusion (Hamilton, Sheets and DeGowin, 1950; Frontali and Stegagno, 1951; Murano, 1955). The improvement in general well-being is striking, the temper of the child improves, growth takes place more normally, bone pains are alleviated and in a child receiving regular transfusions the X-ray evidence of an abnormal bony pattern becomes less marked. Repeated transfusions will in time lead to marked siderosis, but this in the author’s opinion should not be used as an argument against periodic transfusion in patients in whom, without transfusion, the degree of anaemia leads to serious symptoms. Frumin, Waldman and Morris (1952) referred to a child who died, aged 10 years, having received over 76 litres of blood since birth. At necropsy there were all the signs of exogenous hsemochromatosis, with early active portal cirrhosis of the liver (see also Howell and Wyatt (1953) and Ellis, Schulman and Smith (1954)). Fortunately, however, most affected children manage to accommodate themselves remarkably well to haemoglobin levels even as low as 5 g. per 100 ml.; if this is so, they are best left untransfused. Careful studies have been carried out on the effect that transfusion has on the formation of erythrocytes by the recipient. As anaemia is corrected the output of erythrocytes diminishes. This can be demonstrated by the technique of differential agglutination and by using the presence of foetal haemoglobin as a marker of the patient’s own corpuscles (Smith et al., 1955b; Murano, 1955). Because transfusion has an inhibitory effect on erythropoiesis it is probably unwise, in a child receiving repeated 1 With the possible exception of folic acid in rare instances (see p. 211).