Behaviour of Myeloid Precursors in Homozygous β Thalassaemia

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Abstract
Maintaining a high Hb level, through a high transfusion regime, is the best method for treating [human] thalassemia. Not much is known about the effect of this treatment on medullary or extramedullary hemopoiesis, particularly on the extent of erythropoietic inhibition and on the behavior of myelopoiesis. To analyze some aspects of the problem, the myeloid stem cells (CFU-c) in the bone marrow and in the peripheral blood of children with homozygous thalassemia were studied using the agar culture technique. The number of circulating CFU-c observed in 68 patients was higher than in normal subjects. This number was significantly increased after splenectomy. A positive correlation was demonstrated between the number of circulatiqg CFU-c and the time elapsed since the last transfusion. Patients with a high Hb level displayed a marked reduction in the number of CFU-c in their peripheral blood. In 10 patients, before the beginning of transfusions, bone marrow CFU-c were lower than in normal subjects; their number increased after therapy. Most circulating CFU-c were proliferating as shown by the thymidine suicide technique.

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