Agrawal Narendra, Verma Priyanka, Yadav Neha, Ahmed Rayaz, Mehta Pallavi, Soni Priyanka, Francis Shinto, Bhurani Dinesh
Department of Hemato-Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Sector-5, Rohini, New Delhi, 110085 India.
Indian J Hematol Blood Transfus. 2019 Apr;35(2):240-247. doi: 10.1007/s12288-018-1005-2. Epub 2018 Sep 1.
Philadelphia positive ALL (Ph + ALL) is an aggressive leukemia associated with lower remission rates and poor survival. Current treatment approach for Ph + ALL is chemotherapy along with TKI and CNS directed therapy followed by Allogeneic stem cell transplantation (Allo-SCT). To analyze outcome of Ph + ALL with or without Allo-SCT in the era of universal TKI uses. Retrospectively reviewed medical records of 267 patients who were diagnosed and treated for ALL during study period at our centre. Fifty-one Ph + ALL patients (males = 31, females = 20) out of a total of 267 ALL patients were eligible for the study. Post induction 48 patients achieved complete remission while 1 died during induction. Forty-six patients received further treatment with TKI + CNS directed therapy and thereafter the consolidation therapy with Allo-SCT (n = 16) or chemotherapy + TKI (n = 30).Overall mortality was 7/51 (13.9%) (6/16 transplant related mortalities due to GVHD and infections and 1 induction death). Fifteen out of 46 patients (32.6%) had relapse (1/10 relapse after Allo-SCT vs. 14/24 after chemotherapy) on or after consolidation therapy. At a median follow-up of 17.5 months (2-58 months) of cohort, the median EFS was 22 months (95% CI 10.4-33.5 months). The estimated 4 year EFS and PFS in Allo-SCT versus chemotherapy only group was 36.0 ± 17.9 versus 27.3 ± 9.1% ( = 0.21) and 75 ± 21.7 versus 34.1 ± 10.9% ( = 0.02) respectively. Allo-SCT groups has a better progression free survival than chemotherapy group only. Preventing treatment related mortality can further improve outcome after Allo-SCT Ph + ALL.
费城染色体阳性急性淋巴细胞白血病（Ph+ALL）是一种侵袭性白血病，缓解率较低且生存率不佳。目前针对Ph+ALL的治疗方法是化疗联合酪氨酸激酶抑制剂（TKI）以及中枢神经系统定向治疗，随后进行异基因干细胞移植（Allo-SCT）。分析在普遍使用TKI的时代，接受或未接受Allo-SCT的Ph+ALL患者的预后。回顾性分析了在研究期间于本中心诊断并治疗的267例ALL患者的病历。在总共267例ALL患者中，51例Ph+ALL患者（男性31例，女性20例）符合研究条件。诱导缓解后，48例患者达到完全缓解，1例在诱导期死亡。46例患者接受了TKI联合中枢神经系统定向治疗的进一步治疗，此后接受了Allo-SCT巩固治疗（n = 16）或化疗联合TKI巩固治疗（n = 30）。总体死亡率为7/51（13.9%）（6例为与移植相关的死亡，原因是移植物抗宿主病和感染，1例为诱导期死亡）。46例患者中有15例（32.6%）在巩固治疗期间或之后复发（Allo-SCT后复发1/10例，化疗后复发14/24例）。在对该队列进行中位随访17.5个月（2 - 58个月）时，中位无事件生存期（EFS）为22个月（95%置信区间10.4 - 33.5个月）。Allo-SCT组与单纯化疗组的4年EFS估计值分别为36.0±17.9%和27.3±9.1%（P = 0.21），4年无进展生存期（PFS）估计值分别为75±21.7%和34.1±10.9%（P = 0.02）。Allo-SCT组比单纯化疗组具有更好的无进展生存期。预防治疗相关死亡率可进一步改善Allo-SCT治疗Ph+ALL后的预后。
