中文摘要：

目的探讨强化清髓不含胸腺细胞免疫球蛋白的单份非血缘脐血移植治疗伴中枢神经系统白血病（central nervous system leukemia,CNSL）的急性白血病疗效。方法回顾性分析2009年7月至2014年9月在我科接受该治疗的15例急性白血病患者临床特征,其中急性单核细胞白血病2例,急性未分化型白血病1例,急性淋巴细胞白血病12例。所有患者既往化疗过程中存在CNSL,经治疗后移植前CNSL均达完全缓解。全部采用强化清髓预处理方案,其中全身照射/Ara-C/CY方案9例,Ara-c/Bu/CY方案3例,Flu/Bu/CY方案3例,预防移植物抗宿主病（graft-versus-host disease,GVHD）方案为环孢素联合短程霉酚酸酯。结果 14/15例（93.3%）患者获得造血重建,中性粒细胞、血小板植入的中位时间分别为19（13~27）d和41（20~85）d。移植相关并发症：80%（12/15）患者出现植入前综合征;14例可评估的患者中,3例患者出现Ⅱ~Ⅳ度急性GVHD,累积发生率为20.00%,1例患者出现Ⅲ~Ⅳ度急性GVHD,累积发生率为13.33%;3例患者出现局限型皮肤慢性GVHD,移植后720 d累积发生率为20.00%,无广泛型慢性GVHD的发生。所有患者中位随访时间43.2（14.1~76.5）个月,因植入前多脏器功能衰竭死亡1例,重度急性GVHD死亡1例及原发病复发死亡2例;复发3例,其中2例骨髓复发,仅1例CNSL复发,经鞘内注射及头颅放疗,脑脊液恢复正常。3年预计总生存率及无白血病生存率分别为60.0%和53.3%。结论强化清髓不含胸腺细胞免疫球蛋白的单份非血缘脐血移植治疗伴CNSL的急性白血病初步结果显示疗效较好,值得推广应用。

英文摘要：

Objective To evaluate the curative efficacy of single-unit umbilical cord blood transplantation（ s UCBT） without antithymocyte globulin（ ATG） for acute leukemia with central nervous system leukemia（ CNSL） involvement. Methods Retrospective analysis was performed on clinical data of 15 acute leukemia undergoing s UCBT between Jul. 2009 and Sep. 2014 in our department. There were 2 cases of acute mononuclear leukemia,1 case of acute myelocytic leukemia（ M1）,and 12 cases of acute lymphoblastic leukemia. All patients achieved complete remission in CNSL before transplantation. All cases received intensified myeloablative conditioning regimen,with 9 cases of total body irradiation（ TBI） / cytosine arabinoside（ Ara-C） / cyclophosphamide（ CY）,3 cases of Ara-C / Busulfan（ Bu） / CY and 3 cases of Flu / Bu /CY,using a combination of Cs A and MMF for the prophylaxis of graft-versus-host disease（ GVHD）. Results Fourteen patients were engrafted with neutrophils that exceeded 0. 5 × 109/ L in median 19（ 13 ~ 27） d,and had platelet counts ≥20 × 109/ L in median 41（ 20 ~ 85） d. Twelve patients developed pre-engraftment syndrome（ PES）. Among the 14 evaluated cases,3 patients appeared acute GVHD,with the cumulative incidences of Ⅱ ~ Ⅳ and Ⅲ ~ Ⅳ a GVHD of 20. 00% and 13. 33% respectively. There was no extensive chronic GVHD occurred except for 3 cases with skin chronic GVHD in limited type,with a cumulative incidence of 720-day c GVHD of 20. 00%. During the median follow-up of 43. 2（ 14. 1 ~ 76. 5） months,there were 4 patients died,consisting of 1 case with multiple organ failure before engraftment,1 case with severe a GVHD and 2 cases with disease recurrence. Three patients relapsed after transplantation,in which 2 cases of bone marrow relapse. There was only 1 case of central nervous system relapse,and achieved complete remission after intrathecal injection and cranial radiation. The probability of 3-year overall survival rate and disease-free survival rate were 60. 0