Department of Pediatrics, National Taiwan University Hospital, No. 8, Chung-Shan S. Rd., Taipei, Taiwan.
Department of Surgery, National Taiwan University Hospital, No. 7, Chung-Shan S. Rd., Taipei, Taiwan.
J Formos Med Assoc. 2019 Nov;118(11):1537-1545. doi: 10.1016/j.jfma.2018.12.023. Epub 2019 Jan 8.
Post-transplantation lymphoproliferative disorder (PTLD) is a heterogeneous, potentially life-threatening complication after liver transplantation in children. In this study, the disease characteristics, outcomes, and prognostic factors of PTLD were investigated.
A retrospective, observational study was conducted on 16 pediatric liver transplant (LT) recipients who developed PTLD between February 2001 and December 2013 at a tertiary referral center in Taiwan. The disease characteristics and treatment outcomes of the patients were analyzed.
The median time from liver transplantation to the development of PTLD was 8 months. Early onset (<1 year post-transplantation) PTLD constituted 62.5% of the cases. PTLD location was frequently extranodal (81.3%) and mostly involved the gastrointestinal tract (68.8%). Histopathologic findings showed polymorphic PTLD in six cases (37.5%), diffuse large B-cell lymphoma in five cases (31.3%), and Burkitt's PTLD in two cases (12.5%). Early lesions in PTLD, T-cell lymphoma, and Hodgkin's lymphoma were observed in one case each (6.3%). Overall survival at 1- and 5-years post-PTLD diagnosis was 87.5% and 79.5%, respectively. St Jude's classification stage IV disease was associated with poor survival (hazard ratio [HR] = 13.37, P = 0.037). Two patients (12.5%) had chronic rejection after the treatment for PTLD and one patient (6.3%) developed graft failure.
PTLD is a major complication in pediatric LT recipients, but long-term survival is possible in most cases with an adequate treatment strategy. Stage IV disease is a major risk factor for poor survival in pediatric PTLD patients. During the management of PTLD, the possibility of chronic rejection and even graft failure should be considered.
移植后淋巴组织增生性疾病（PTLD）是儿童肝移植后一种具有异质性、潜在威胁生命的并发症。本研究旨在探讨 PTLD 的疾病特征、结局和预后因素。
对 2001 年 2 月至 2013 年 12 月在台湾一家三级转诊中心接受肝移植后发生 PTLD 的 16 例儿科肝移植（LT）受者进行回顾性、观察性研究。分析患者的疾病特征和治疗结局。
从肝移植到发生 PTLD 的中位时间为 8 个月。早期发病（移植后<1 年）占 62.5%。PTLD 部位多为结外（81.3%），以胃肠道受累最常见（68.8%）。组织病理学检查显示 6 例（37.5%）为多形性 PTLD，5 例（31.3%）为弥漫性大 B 细胞淋巴瘤，2 例（12.5%）为伯基特淋巴瘤。PTLD、T 细胞淋巴瘤和霍奇金淋巴瘤的早期病变各 1 例（6.3%）。PTLD 诊断后 1 年和 5 年的总生存率分别为 87.5%和 79.5%。圣裘德分类分期为 IV 期疾病与生存率差相关（危险比[HR] = 13.37，P = 0.037）。2 例（12.5%）患者在治疗 PTLD 后发生慢性排斥反应，1 例（6.3%）患者发生移植物失功。
PTLD 是儿科 LT 受者的主要并发症，但大多数患者通过适当的治疗策略可获得长期生存。IV 期疾病是儿科 PTLD 患者生存不良的主要危险因素。在管理 PTLD 时，应考虑发生慢性排斥反应甚至移植物失功的可能。
