Bacigalupo A, Frassoni F, Van Lint M T, Occhini D, Pittaluga P A, Repetto M, Piaggio G, Sessarego M, Caimo A, Congiu A
Cancer. 1986 Nov 15;58(10):2307-11. doi: 10.1002/1097-0142(19861115)58:10<2307::aid-cncr2820581025>3.0.co;2-h.
Thirty patients with chronic granulocytic leukemia (CGL), were given cyclophosphamide 60 mg/kg on each of 2 consecutive days, followed by total body irradiation (TBI) 10 Gy and an HLA-identical bone marrow transplant (BMT). Eleven patients were in the accelerated phase of their disease (CGLacc) or in second/secondary chronic phase (CGL-2CP), with a median age of 33 years: four patients died of transplant related complications, and four of recurrent leukemia; three patients are alive and well 19, 31, 33 months from BMT. The actuarial 33-month survival is 27%. The actuarial relapse rate is 50%. Nineteen patients were in their first chronic phase (1CP), with a median age of 32 years: three died of graft versus host disease (GvHD), two of infection, and two of acute respiratory distress syndrome (ARDS); 12 are alive and well 6 to 29 months post-BMT. The actuarial 29-month survival is 63%. The actuarial survival of patients younger than 30 years is 63%, compared to 62% for patients older than 30 (P = 0.1). The survival of patients grafted within or after 24 months from the onset of CGL is respectively 87% and 45% (P = 0.04). None of the patients grafted in 1CP had a true hematologic-cytogenetic relapse. The Ph' chromosome was detected on one occasion in two patients 12, 13 months post-BMT: they both remain hematologically normal and Ph1-negative 3 to 6 months later, after discontinuation of cyclosporin A. This study confirms that survival exceeding 60% can be obtained in CGL in the first chronic phase, whereas less than 30% of patients will survive if grafted in accelerated, second/secondary chronic phase, mainly because of leukemic relapse. The duration of the disease seems to be relevant to the outcome of the transplant. The effect of post-transplant immunosuppression, in our case cyclosporin A, on the interaction between normal and Ph1-positive hemopoietic cells, may deserve further attention.
30例慢性粒细胞白血病（CGL）患者，连续2天每天接受60mg/kg环磷酰胺治疗，随后进行10Gy全身照射（TBI）及 HLA 相同的骨髓移植（BMT）。11例患者处于疾病加速期（CGLacc）或第二次/继发慢性期（CGL - 2CP），中位年龄33岁：4例死于移植相关并发症，4例死于白血病复发；3例患者在BMT后19、31、33个月存活且状况良好。33个月的精算生存率为27%。精算复发率为50%。19例患者处于首次慢性期（1CP），中位年龄32岁：3例死于移植物抗宿主病（GvHD），2例死于感染，2例死于急性呼吸窘迫综合征（ARDS）；12例患者在BMT后6至29个月存活且状况良好。29个月的精算生存率为63%。年龄小于30岁患者的精算生存率为63%，30岁以上患者为62%（P = 0.1）。CGL发病后24个月内或24个月后接受移植的患者生存率分别为87%和45%（P = 0.04）。处于1CP接受移植的患者均未出现真正的血液学 - 细胞遗传学复发。2例患者在BMT后12、13个月有一次检测到Ph'染色体：在停用环孢素A后3至6个月，他们血液学均保持正常且Ph1阴性。本研究证实，处于首次慢性期的CGL患者可获得超过60%的生存率，而在加速期、第二次/继发慢性期接受移植的患者生存率不到30%，主要原因是白血病复发。疾病持续时间似乎与移植结果相关。在我们的研究中，移植后免疫抑制药物环孢素A对正常和Ph1阳性造血细胞之间相互作用的影响，可能值得进一步关注。
