Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
Primary CNS Posttransplant Lymphoproliferative Disease (PTLD): An International Report of 84 Cases in the Modern Era
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DOI：
10.1111/ajt.12211
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发表时间：
2013-06-01
影响因子：
8.8
通讯作者：
Trappe, R.
中科院分区：
文献类型：
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作者：
Evens, A. M.;Choquet, S.;Trappe, R.
我们对实体器官移植(SOT)相关的初级中枢神经系统(PCNS)移植后淋巴增生性疾病(PTLD)进行了多中心的国际分析。在84例PCNS PTLD患者中，SOT转PTLD的中位时间为54个月，79%为肾脏SOT，83%为组织学单形性，94%为EBV+。此外，33%的人有脑深部受累，10%的人有脑脊液受累，而没有眼部疾病。免疫抑制减少了93%；其他一线治疗包括大剂量甲氨蝶呤(48%)、大剂量阿糖胞苷(33%)、脑放疗(24%)和/或利妥昔单抗(44%)。总有效率为60%，而与治疗相关的死亡率为13%。中位随访期42个月，3年无进展生存率(PFS)和总生存率(OS)分别为32%和43%。对于接受利妥昔单抗和/或大剂量阿糖胞苷治疗的患者，有改善PFS的单变量分析的趋势。在多变量COX回归分析中，功能状态差预示预后差(HR2.61，95%CI1.32~5.17，p=0.006)，而LDH升高预示OS差(HR4.16，95%CI1.29~13.46，p=0.02)。此外，在多变量分析中，对一线治疗缺乏反应是最主要的预后因素(HR8.70，95%CI2.56-29.57，p=0.0005)。总之，PCNS PTLD似乎代表了PTLD谱系中的一个独特的临床病理实体，与肾脏SOT相关，发生较晚，是单形性的，并保持EBV阳性。
We performed a multicenter, International analysis of solid organ transplant (SOT)-related primary central nervous system (PCNS) posttransplant lymphoproliferative disease (PTLD). Among 84 PCNS PTLD patients, median time of SOT-to-PTLD was 54 months, 79% had kidney SOT, histology was monomorphic in 83% and tumor was EBV+ in 94%. Further, 33% had deep brain involvement, 10% had CSF involvement, while none had ocular disease. Immunosuppression was reduced in 93%; additional first-line therapy included high-dose methotrexate (48%), high-dose cytarabine (33%), brain radiation (24%) and/or rituximab (44%). The overall response rate was 60%, while treatment-related mortality was 13%. With 42-month median follow-up, three-year progression-free survival (PFS) and overall survival (OS) were 32% and 43%, respectively. There was a trend on univariable analysis for improved PFS for patients who received rituximab and/or high-dose cytarabine. On multivariable Cox regression, poor performance status predicted inferior PFS (HR 2.61, 95% CI 1.32-5.17, p=0.006), while increased LDH portended inferior OS (HR 4.16, 95% CI 1.29-13.46, p=0.02). Moreover, lack of response to first-line therapy was the most dominant prognostic factor on multivariable analysis (HR 8.70, 95% CI 2.56-29.57, p=0.0005). Altogether, PCNS PTLD appears to represent a distinct clinicopathologic entity within the PTLD spectrum that is associated with renal SOT, occurs late, is monomorphic and retains EBV positivity.