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.

    254/396 (page 212)
    25 mg. per 100 ml. after splenectomy. Das Gupta and his colleagues reported essentially similar findings; 13 patients with the Cooley trait had values of 0-6^-3 mg. per 100 ml., and 22 patients with Cooley’s anaemia values of 4-3-56-5 mg. per 100 ml. Substantially similar figures based on a larger series of patients were given by Das Gupta and his co-workers (1958). Serum Proteins. There were studied, using paper electrophoresis, by Allamanis (1955) in 36 children with Cooley’s anaemia: in 11 infants less than 24 months old the patterns were almost normal; in older children albumin was decreased (on an average by 52%), and y globulin increased (on an average by 30%); there were minor reductions in the concentrations of a 1; a 2 an d /5 globulins, and the flocculation tests often gave abnormal results. Increases in the plasma and corpuscular lipids and phospholipids were described by Erickson and co-workers (1937) and Schwarz-Tiene, Corda and Careddu (1953), the latter authors also reporting low levels of free and combined cholesterol. Recently, Choremis, Zannos and Basti (1957) have reported finding in 20 infants and children with Cooley’s anaemia that the output of amino acids in the urine was markedly increased. The significance and cause of this abnormality are obscure. Urobilinogen Excretion. This is increased, and as in pernicious anaemia it is probable that not all the faecal urobilinogen is derived from erythrocytes which have circulated in the blood stream. Sturgeon and Finch (1957) observed excretions of 400-600 mg. per day in three children, which were about ten times the anticipated amount. Pathology Bone Marrow in Thalassaemia. The bone marrow is hyperplastic, the degree of hyperplasia varying directly with the severity of the anaemia. The hyperplasia is the result of active erythropoiesis, and in severe cases the erythroid : myeloid ratio may exceed unity. Erythropoiesis is typically normoblastic. There is a tendency for the developing normoblasts to be smaller than normal (micronormoblasts) (Fig. 90); this is mostly due to diminution in the amount of cytoplasm and is most marked in the most ripened cells. Detailed measurements are given by Astaldi, Tolentino and Sacchetti (1951a). In thalassaemia major the percentage of basophilic normoblasts is often unusually high; in thalassaemia minor polychromatic and pyknotic normoblasts predominate. Astaldi and Tolentino (1952) claimed that in the most serious cases of thalassaemia major there was some delay in the enucleation of the orthochromatic normoblasts. Megaloblastic change, as the result of folic-acid deficiency, has already been referred to as rather a rare event (Fig. 89). In severe cases haemoglobin may appear to be formed in a patchy fashion in the cytoplasm of the developing normoblasts,