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异基因造血干细胞移植治疗伴有中枢神经系统受累的 ATL:长崎移植组的经验。

Allogeneic hematopoietic stem cell transplantation for ATL with central nervous system involvement: the Nagasaki transplant group experience.

机构信息

Department of Hematology, Atomic Bomb Disease and Hibakusya Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

Sasebo City General Hospital, Sasebo, Japan.

出版信息

Int J Hematol. 2011 Oct;94(4):390-394. doi: 10.1007/s12185-011-0935-3. Epub 2011 Sep 30.

Abstract

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is regarded as a curative option for aggressive adult T cell leukemia-lymphoma (ATL). However, the efficacy and safety of allo-HSCT for ATL with central nervous system (CNS) involvement, which is highly resistant to chemotherapy, remain controversial. We analyzed 10 ATL patients with CNS involvement who received allo-HSCT at three institutions in Nagasaki prefecture between 2000 and 2007. The 3-year overall survival rate was 40%, and the median observation time of the four surviving patients was 1532 days (range 945-2212 days). Two of four surviving patients received highly intensive local treatment for the CNS; one with 26 intrathecal injections of antineoplastic agents, and the other with whole cerebrospinal irradiation before transplantation. However, the other two patients received conventional or reduced-intensity conditioning with standard intrathecal chemotherapy. Three of the four surviving patients experienced chronic GVHD, and two of three patients with grade 3 or 4 acute GVHD were free from CNS relapse. From these data, it seems that both intensive local treatment for CNS disease and systemic GVHD contributed to the long-term control of CNS involvement. Although our data suggest that allo-HSCT is a therapeutic option for ATL with CNS disease, high transplant-related mortality (six cases) indicates the need for further studies to develop more effective procedures for CNS disease, and to reduce transplant-related morbidity.

摘要

异基因造血干细胞移植(allo-HSCT)被认为是侵袭性成人 T 细胞白血病/淋巴瘤(ATL)的一种根治性选择。然而,对于中枢神经系统(CNS)受累的 ATL,其疗效和安全性仍存在争议,因为这种疾病对化疗具有高度耐药性。我们分析了 2000 年至 2007 年间在长崎县三家机构接受 allo-HSCT 的 10 例 CNS 受累 ATL 患者。3 年总生存率为 40%,4 例存活患者的中位观察时间为 1532 天(范围 945-2212 天)。4 例存活患者中有 2 例接受了高强度的 CNS 局部治疗;1 例接受了 26 次鞘内注射抗肿瘤药物,另 1 例在移植前接受了全脑脊髓照射。然而,另外 2 例患者接受了常规或低强度预处理联合标准鞘内化疗。4 例存活患者中有 3 例发生慢性移植物抗宿主病,3 例中有 2 例 3 级或 4 级急性移植物抗宿主病的患者无 CNS 复发。从这些数据来看,CNS 疾病的强化局部治疗和全身移植物抗宿主病似乎都有助于长期控制 CNS 受累。尽管我们的数据表明 allo-HSCT 是治疗 CNS 疾病的 ATL 的一种治疗选择,但高移植相关死亡率(6 例)表明需要进一步研究以开发更有效的 CNS 疾病治疗方法,并降低移植相关发病率。

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