CNS or bone marrow involvement as risk factors for poor survival in post-transplantation lymphoproliferative disorders in children after solid organ transplantation
CNS or bone marrow involvement as risk factors for poor survival in post-transplantation lymphoproliferative disorders in children after solid organ transplantation
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DOI：
10.1200/jco.2006.10.2392
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发表时间：
2007-11-01
影响因子：
45.3
通讯作者：
Klein, Christoph
中科院分区：
文献类型：
--
作者：
Maecker, Britta;Jack, Thomas;Klein, Christoph
目的确定生存的预后因素在儿科移植后淋巴增生性疾病（PTLD）后实体organ transplantation.Patients和方法一个多中心，回顾性病例分析的55名儿童实体器官移植受体（肾，肝，心/肺）发展PTLD报告德国儿科PTLD注册。分析患者病历的肿瘤特征（组织学、免疫表型、细胞遗传学、EB病毒[EBV]检测）、分期、治疗和结局。使用单变量和考克斯回归analysis.Results PTLD诊断的中位时间为29个月后器官移植，与一个显着较短的滞后时间在肝脏（0.83年）与心脏或肾移植受体（3.33和3.10年，分别P = 0.001）的总体和无事件生存的概率进行了分析。5年总生存率和无事件生存率分别为68%和59%，59%的患者生存10年。伴有骨髓和/或中枢神经系统受累的IV期疾病与生存率低独立相关（P = .0005）。早发型和晚发型PTLD、单型或多型PTLD、EBV阳性或EBV阴性PTLD之间的结局无差异。结论IV期疾病是PTLD患儿生存不良的独立危险因素。需要前瞻性多中心试验来描述其他的危险因素，并评估儿科PTLD的治疗方法。
Purpose To identify prognostic factors of survival in pediatric post-transplantation lymphoproliferative disorder (PTLD) after solid organ transplantation.Patients and Methods A multicenter, retrospective case analysis of 55 pediatric solid organ graft recipients (kidney, liver, heart/lung) developing PTLD were reported to the German Pediatric-PTLD registry. Patient charts were analyzed for tumor characteristics (histology, immunophenotypes, cytogenetics, EpsteinBarr virus [EBV] detection), stage, treatment, and outcome. Probability of overall and event-free survival was analyzed in defined subgroups using univariate and Cox regression analyses.Results PTLD was diagnosed at a median time of 29 months after organ transplantation, with a significantly shorter lag time in liver (0.83 years) versus heart or renal graft recipients (3.33 and 3.10 years, respectively; P = .001). The 5-year overall and event-free survival was 68% and 59%, respectively, with 59% of patients surviving 10 years. Stage IV disease with bone marrow and/ or CNS involvement was associated independently with poor survival (P = .0005). No differences in outcome were observed between early- and late- onset PTLD, monomorphic or polymorphic PTLD, and EBV- positive or EBV-negative PTLD, respectively. Patients with Burkitt or Burkitt-like PTLD and c-myc translocations had short survival (< 1 year).Conclusion Stage IV disease is an independent risk factor for poor survival in pediatric PTLD patients. Prospective multicenter trials are needed to delineate additional risk factors and to assess treatment approaches for pediatric PTLD.