Prati D
Centro Trasfusionale e di Immunologia dei Trapianti, IRCCS Ospedale Maggiore di Milano, Italia.
Vox Sang. 2000;79(3):129-37. doi: 10.1159/000031230.
Early and regular blood transfusion therapy in patients with homozygous beta-thalassaemia decreases the complications of severe anaemia and prolongs survival. In the long term, however, the beneficial effects of transfusions are limited by the organ damage resulting from iron overload, a consequence of the body's limited capacity to excrete iron, and by the complications of infection with blood-borne agents. Transfusion regimens for beta-thalassaemia have changed substantially during the past four decades. In current protocols, pre-transfusion haemoglobin concentration should not exceed 95 g/l. This allows adequate control of anaemia, with a relatively low rate of iron accumulation. Although iron chelation therapy has successfully improved survival free from cardiac disease, thalassaemic patients continuously present new clinical challenges. In fact, the vast majority of them suffer from post-transfusion chronic hepatitis C, which is expected to significantly contribute to morbidity in the forthcoming years. Furthermore, recent studies demonstrated that thalassaemics are at high risk of acquiring several blood-borne viruses. The potential role of these multiple infections in inducing clinical disease is still uncertain, and needs to be thoroughly clarified in future surveys.
对纯合子β地中海贫血患者进行早期和定期输血治疗可减少严重贫血的并发症并延长生存期。然而，从长远来看，输血的有益效果受到铁过载导致的器官损害的限制，铁过载是身体排泄铁的能力有限的结果，同时也受到血源性病原体感染并发症的限制。在过去的四十年中，β地中海贫血的输血方案发生了很大变化。在当前方案中，输血前血红蛋白浓度不应超过95 g/l。这可以充分控制贫血，铁积累率相对较低。尽管铁螯合疗法已成功提高了无心脏病患者的生存率，但地中海贫血患者仍不断面临新的临床挑战。事实上，他们中的绝大多数患有输血后慢性丙型肝炎，预计在未来几年中这将对发病率产生重大影响。此外，最近的研究表明，地中海贫血患者感染多种血源性病原体的风险很高。这些多重感染在诱发临床疾病中的潜在作用仍不确定，需要在未来的调查中彻底阐明。
