Zhu Y X, Zhu M Q, Dai H P, Liu S N, Yin J, Li Z, Cui Q Y, Zhu X M, Wu D P, Tang X W
Jiangsu Institute of Hematology, First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Haemostasis, Suzhou 215006, China.
Zhonghua Xue Ye Xue Za Zhi. 2019 Dec 14;40(12):1021-1025. doi: 10.3760/cma.j.issn.0253-2727.2019.12.010.
Early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) is a recently recognized high-risk T lymphoblastic leukemia subgroup. The optimal therapeutic approaches to adult patients with ETP-ALL are poorly characterized. In this study, we explore the efficacy and outcome of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for ETP-ALL. The clinical data of 23 patients with ETP-ALL receiving allo-HSCT from 2010 to 2018 were retrospectively analyzed. Patients with ETP-ALL were diagnosed based on the characteristic immunophenotypes. Second-generation sequencing was done in all patients. As to the donors, 12 patients had haploidentical donors (Haplo-HSCT) , 7 HLA-matched sibling donors (Sib-HSCT) and 4 HLA-matched unrelated donors (URD-HSCT) . Before transplantation, 19 patients achieved complete remission (CR) and 4 patients without. The main clinical features of ETP-ALL included high white blood cell counts in 5 patients, splenomegaly in 14, lymphadenopathy in 19, and thymus masses in 5. According to cytogenetic and molecular characteristics, 11 patients had gene mutations related to myeloid tumors, and 7 with high risk Karyotype. After first induction regimen, 14/23 patients achieved CR. 5 patients reached CR after more than 2 cycles of chemotherapy, while another 4 patients did not reach CR. After allo-HSCT, 22 patients were successfully implanted. The median time of granulocyte and platelet reconstitution was +12 and +19 days. One patient died of transplant-related infection at +14 days. The estimated 18-month overall survival (OS) and relapse-free survival (RFS) rates were (55.0±14.4) % and (48.1±14.7) % respectively. Transplant-related mortality was 4.3%. The median OS in patients achieving CR before transplantation was 20 months, however, that in patients without CR was only 13 months. OS and RFS between haplo-HSCT and sib-HSCT were comparable (=0.460 and 0.420 respectively) . Allo-HSCT is an effective therapy in some patients with ETP-ALL. Salvage HSCT cannot overcome the poor outcome. Haplo-HSCT and sib-HSCT in ETP-ALL patients have the similar clinical outcome.
早期T细胞前体急性淋巴细胞白血病（ETP-ALL）是最近才被认识的高危T淋巴细胞白血病亚组。针对成年ETP-ALL患者的最佳治疗方法目前尚不明确。在本研究中，我们探讨了异基因造血干细胞移植（allo-HSCT）治疗ETP-ALL的疗效和结局。回顾性分析了2010年至2018年期间接受allo-HSCT的23例ETP-ALL患者的临床资料。ETP-ALL患者根据其特征性免疫表型进行诊断。所有患者均进行了二代测序。关于供者，12例患者有单倍型相合供者（单倍型HSCT），7例有HLA配型相合的同胞供者（同胞HSCT），4例有HLA配型相合的无关供者（URD-HSCT）。移植前，19例患者达到完全缓解（CR），4例未达到。ETP-ALL的主要临床特征包括5例白细胞计数高，14例脾肿大，19例淋巴结肿大，5例胸腺肿块。根据细胞遗传学和分子特征，11例患者有与髓系肿瘤相关的基因突变，7例有高危核型。首次诱导方案后，23例患者中有14例达到CR。5例患者在2个以上化疗周期后达到CR，而另外4例患者未达到CR。allo-HSCT后，22例患者成功植入。粒细胞和血小板重建的中位时间分别为+12天和+19天。1例患者在+14天时死于移植相关感染。估计18个月总生存率（OS）和无复发生存率（RFS）分别为（55.0±14.4）%和（48.1±14.7）%。移植相关死亡率为4.3%。移植前达到CR的患者中位OS为20个月，而未达到CR的患者仅为13个月。单倍型HSCT和同胞HSCT之间的OS和RFS相当（分别为=0.460和0.420）。allo-HSCT对部分ETP-ALL患者是一种有效的治疗方法。挽救性HSCT无法克服不良结局。ETP-ALL患者的单倍型HSCT和同胞HSCT临床结局相似。
