Gabutti V, Miniero R, Piga A, Incarbone E, Sacchetti L, Balegno G
Br J Haematol. 1980 Aug;45(4):599-605. doi: 10.1111/j.1365-2141.1980.tb07183.x.
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.
通过高输血方案维持高血红蛋白水平是治疗地中海贫血的最佳方法。关于这种治疗对髓内或髓外造血的影响，特别是对红细胞生成抑制程度和骨髓生成行为的影响，人们了解得并不多。为了分析该问题的某些方面，我们采用琼脂培养技术，研究了纯合子地中海贫血患儿骨髓和外周血中的髓系干细胞（CFU-c）。在68例患者中观察到的循环CFU-c数量高于正常受试者。脾切除术后，这一数量显著增加。循环CFU-c数量与上次输血后经过的时间之间呈正相关。血红蛋白水平高的患者外周血中CFU-c数量明显减少。在10例患者中，输血开始前骨髓CFU-c低于正常受试者；治疗后其数量增加。如用胸苷自杀技术所示，大多数循环CFU-c正在增殖。
