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