Tichelli A, Gratwohl A, Würsch A, Nissen C, Speck B
Department of Internal Medicine, Kantonsspital, Basel, Switzerland.
Br J Haematol. 1988 Jul;69(3):413-8. doi: 10.1111/j.1365-2141.1988.tb02382.x.
137 patients with severe aplastic anaemia (SAA) were treated in Basel from 1976 to 1986. 34 underwent bone marrow transplantation (BMT) and 103 received antilymphocyte globulin (ALG) therapy. We have analysed the incidence of late haematological complications in both groups of patients. 20 patients treated with ALG developed a late haematological complication. A myelodysplastic syndrome or frank leukaemia occurred in eight and paroxysmal nocturnal haemoglobinuria (PNH) in 13 patients. Nine of the 13 patients with PNH had clinical signs of haemolysis, four only had positive laboratory tests. One patient had PNH and acute leukaemia. The risk of developing a haematological complication increased continuously and reached 57% at 8 years. Neither PNH nor leukaemia occurred in patients treated with BMT. The increased survival rate and the long observation time after ALG therapy have revealed a new perspective of the prognosis of aplastic anaemia. Patients treated with BMT appear to be cured whereas those treated with ALG remain at risk for late complications.
1976年至1986年期间，巴塞尔对137例重型再生障碍性贫血（SAA）患者进行了治疗。34例接受了骨髓移植（BMT），103例接受了抗淋巴细胞球蛋白（ALG）治疗。我们分析了两组患者晚期血液学并发症的发生率。接受ALG治疗的20例患者出现了晚期血液学并发症。8例发生骨髓增生异常综合征或明显白血病，13例发生阵发性夜间血红蛋白尿（PNH）。13例PNH患者中有9例有溶血的临床体征，4例仅有实验室检查阳性。1例患者同时患有PNH和急性白血病。发生血液学并发症的风险持续增加，8年时达到57%。接受BMT治疗的患者未发生PNH或白血病。ALG治疗后生存率的提高和较长的观察时间揭示了再生障碍性贫血预后的新观点。接受BMT治疗的患者似乎已治愈，而接受ALG治疗的患者仍有发生晚期并发症的风险。
