Fu Li, Xie Jianlan, Lin Jun, Wang Jingshi, Wei Na, Huang Dayong, Wang Tingting, Shen Jing, Zhou Xiaoge, Wang Zhao
Capital Medical University, Beijing Friendship Hospital, Beijing, China.
Indian J Hematol Blood Transfus. 2017 Dec;33(4):492-499. doi: 10.1007/s12288-017-0799-7. Epub 2017 Mar 3.
Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous group of lymphoid neoplasms associated with immunosuppression following transplantation. Among PTLDs, monomorphic PTLD (m-PTLD) is the largest category; however, its characteristics and survival outcome are not fully understood because of low incidence. This study enrolled 30 adult patients with m-PTLD after kidney-transplantation (KT, n = 17) and hematopoietic stem cell transplantation (HSCT, n = 13) from January 1998 to December 2014. The incidence rates of m-PTLD were 0.74 and 3.63% in the KT and HSCT groups, respectively. M-PTLD patients in the HSCT group were younger and showed earlier onset, with EBV-encoded small RNAs (EBER) more frequently identified. Diffuse large B cell lymphoma (DLBCL) was the main pathological type, and the digestive system was the most extranodal involvement site in m-PTLD after KT and HSCT. Among the 28 patients with DLBCL m-PTLD,the complete remission rate after rituximab treatment was higher than in patients not administered rituximab treatment ( = 0.038). With a median follow-up of 46 months after m-PTLD diagnosis, the estimated 5-year overall survival (OS) was 59.2 ± 9.1% in all patients, and 64.2 ± 11.8 and 52.7 ± 14.1% in the KT and HSCT groups, respectively ( = 0.741). ECOG PS, Ann Arbor stage, and CD68 IHC expression were independent prognostic factors for OS. M-PTLD is a rare but serious complication after transplantation. Ongoing efforts to standardize safe and effective treatment protocols would improve the poor overall survival. The independent prognostic factors contributed to risk-stratified treatment, and might be validated by larger studies.
移植后淋巴细胞增殖性疾病（PTLDs）是一组异质性的淋巴样肿瘤，与移植后的免疫抑制相关。在PTLDs中，单形性PTLD（m-PTLD）是最大的类别；然而，由于其发病率低，其特征和生存结果尚未完全明确。本研究纳入了1998年1月至2014年12月期间30例肾移植（KT，n = 17）和造血干细胞移植（HSCT，n = 13）后发生m-PTLD的成年患者。m-PTLD在KT组和HSCT组中的发病率分别为0.74%和3.63%。HSCT组中的m-PTLD患者更年轻，发病更早，更频繁地检测到EBV编码的小RNA（EBER）。弥漫性大B细胞淋巴瘤（DLBCL）是主要的病理类型，消化系统是KT和HSCT后m-PTLD最常累及的结外部位。在28例DLBCL m-PTLD患者中，利妥昔单抗治疗后的完全缓解率高于未接受利妥昔单抗治疗的患者（P = 0.038）。在m-PTLD诊断后中位随访46个月时，所有患者的估计5年总生存率（OS）为59.2±9.1%，KT组和HSCT组分别为64.2±11.8%和52.7±14.1%（P = 0.741）。ECOG体能状态、Ann Arbor分期和CD68免疫组化表达是OS的独立预后因素。m-PTLD是移植后一种罕见但严重的并发症。持续努力规范安全有效的治疗方案将改善较差的总生存率。这些独立的预后因素有助于进行风险分层治疗，可能需要更大规模的研究来验证。
