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韩国异基因造血干细胞移植后移植后淋巴细胞增殖性疾病的临床特征及预后

Clinical characteristics and outcomes of posttransplant lymphoproliferative disorders following allogeneic hematopoietic stem cell transplantation in Korea.

作者信息

Park Sun Hee, Choi Su Mi, Lee Dong Gun, Choi Jung Hyun, Yoo Jin Hong, Kim Hee Je, Kim Dong Wook, Lee Jong Wook, Min Woo Sung, Shin Wan Shik, Kim Chun Choo

机构信息

Department of Internal Medicine, The Catholic Hematopoietic Stem Cell Transplantation Center, The Catholic University of Korea, College of Medicine, Seoul, Korea.

出版信息

J Korean Med Sci. 2006 Apr;21(2):259-64. doi: 10.3346/jkms.2006.21.2.259.

Abstract

Between 1995 and 2003, seven cases of posttransplant lymphoproliferative disorder (PTLD) were identified among 1,116 patients who received allogeneic hematopoietic stem cell transplantations (HSCT) at Catholic HSCT Center (overall incidence 0.6%). Five (71.4%) patients had episodes of acute graft-versus-host-disease (GVHD) and were treated with steroids. Cervical lymphadenopathy was observed in most cases (71.4%), but clinical symptoms varied depending on the involved sites. Pathologic findings varied: 1 case of plasmacytic hyperplasia, 3 of polymorphic PTLD, 2 of diffuse large B-cell lymphoma, 1 of large T-cell lymphoma, which proved to be associated with Epstein-Barr virus (EBV). The proportion of EBV-negative PTLD was 33.3%. Five patients demonstrated a good response to treatment (treatment response rate 71.4%). The overall mortality was 42.8%, and one death was directly attributable to PTLD. The incidence of PTLD is expected to increase, based on the rising use of grafts from alternative donors and recent clinical features of PTLD manifested by a disseminated and fulminant nature. It is necessary to have a high level of suspicion when monitoring patients and readily adopt prompt and effective cellular immunotherapy for PTLD.

摘要

1995年至2003年期间,在天主教造血干细胞移植中心接受异基因造血干细胞移植(HSCT)的1116例患者中,确诊了7例移植后淋巴细胞增殖性疾病(PTLD)(总发病率0.6%)。5例(71.4%)患者发生急性移植物抗宿主病(GVHD)并接受了类固醇治疗。大多数病例(71.4%)观察到颈部淋巴结病,但临床症状因受累部位而异。病理结果各不相同:1例浆细胞增生,3例多形性PTLD,2例弥漫性大B细胞淋巴瘤,1例大T细胞淋巴瘤,均被证实与EB病毒(EBV)有关。EBV阴性PTLD的比例为33.3%。5例患者对治疗反应良好(治疗有效率71.4%)。总死亡率为42.8%,1例死亡直接归因于PTLD。基于替代供体移植物使用的增加以及PTLD近期表现出的播散性和暴发性临床特征,预计PTLD的发病率将会上升。在监测患者时必须高度怀疑,并对PTLD及时采取迅速有效的细胞免疫治疗。

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