Behaviour of myeloid precursors in homozygous beta thalassaemia.
Maintaining a high haemoglobin level, through a high transfusion regime, is the best method for treating thalassaemia. Not much is known about the effect of this treatment on medullary or extramedullary haemopoiesis, particularly on the extent of erythropoietic inhibition and on the behaviour of myelopoiesis. In order to analyse some aspects of the problem, we studied the myeloid stem cells (CFU-c) in the bone marrow and in the peripheral blood of children with homozygous thalassaemia, 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 circulating 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.
D000293:Adolescent; D001803:Blood Transfusion; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003114:Colony-Forming Units Assay; D006412:Hematopoietic Stem Cells / Q000473:pathology*; D006452:Hemoglobinometry; D006801:Humans; D007223:Infant; D013156:Splenectomy; D013789:Thalassemia / Q000097:blood / Q000473:pathology* / Q000628:therapy; D013997:Time Factors