Outcome of Philadelphia Positive Acute Lymphoblastic Leukemia With or Without Allogeneic Stem Cell Transplantation in a Retrospective Study

Outcome of Philadelphia Positive Acute Lymphoblastic Leukemia With or Without Allogeneic Stem Cell Transplantation in a Retrospective Study
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DOI:
10.1007/s12288-018-1005-2
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发表时间:
2019-04-01
影响因子:
0.9
通讯作者:
Bhurani, Dinesh
Bhurani, Dinesh
中科院分区:
医学4区
文献类型:
--
作者:
Agrawal, Narendra;Verma, Priyanka;Bhurani, Dinesh

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费城阳性 ALL (Ph+ALL) 是一种侵袭性白血病,与较低的缓解率和较差的生存率相关。目前 Ph+ALL 的治疗方法是化疗结合 TKI 和 CNS 定向治疗,然后进行同种异体干细胞移植 (Allo-SCT)。分析在通用 TKI 使用时代,有或没有 Allo-SCT 的 Ph+ALL 的结果。回顾性审查了研究期间在我们中心诊断和治疗 ALL 的 267 名患者的医疗记录。总共 267 名 ALL 患者中,有 51 名 Ph+ALL 患者(男性 = 31,女性 = 20)有资格参加该研究。诱导后 48 名患者获得完全缓解,而 1 名患者在诱导期间死亡。 46 名患者接受了 TKI+CNS 定向治疗的进一步治疗,随后采用 Allo-SCT(n=16)或化疗+TKI(n=30)巩固治疗。总死亡率为 7/51(13.9%)(6/16 因 GVHD 和感染导致的移植相关死亡率以及 1 例诱导死亡)。 46 名患者中有 15 名 (32.6%) 在巩固治疗期间或之后出现复发(Allo-SCT 后复发 1/10,化疗后复发 14/24)。在队列中位随访 17.5 个月(2-58 个月)时,中位 EFS 为 22 个月(95% CI 10.4-33.5 个月)。 Allo-SCT 与单纯化疗组的估计 4 年 EFS 和 PFS 分别为 36.0 +/- 17.9 与 27.3 +/- 9.1% (p=0.21) 和 75 +/- 21.7 与 34.1 +/- 10.9% (p=0.02)。 Allo-SCT 组比仅化疗组具有更好的无进展生存期。预防治疗相关的死亡率可以进一步改善 Allo-SCT Ph+ALL 后的结果。
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 17.5months (2-58months) of cohort, the median EFS was 22months (95% CI 10.4-33.5months). The estimated 4year EFS and PFS in Allo-SCT versus chemotherapy only group was 36.0 +/- 17.9 versus 27.3 +/- 9.1% (p=0.21) and 75 +/- 21.7 versus 34.1 +/- 10.9% (p=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.