From the Department of Pediatric Hematology and Oncology, Baskent University Medical Faculty, Ankara, Turkey.
Exp Clin Transplant. 2024 Oct;22(Suppl 5):35-40. doi: 10.6002/ect.pedsymp2024.O5.
Posttransplant lymphoproliferative disorder is a consequential complication following solid-organ transplant, particularly associated with the Epstein-Barr virus. We studied a single center's cases of pediatric posttransplant lymphoproliferative disorder for a 7-year period and focused on incidence rates, anatomic sites involved, and correlation with clinical outcomes. We explored clinical features and treatment outcomes in patients with pediatric posttransplant lymphoproliferative disorder, with emphasis on patient survival and associated clinical ramifications.
This was a retrospective analysis of medical records from pediatric solid-organ transplant recipients (liver or kidney) at Baskent University Ankara Hospital Organ Transplantation Center between January 1, 2017, and January 1, 2024, approved by the Institutional Review Board (KA24/63). We identified cases based on pathology-confirmed persistent lymphadenopathy or tumorous lesions. Patient categorization distinguished between malignant and benign groups. Early posttransplant lymphoproliferative disorder was defined within the initial year after transplant. Epstein?Barr virus association was determined through in situ hybridization, and patient characteristics were reviewed comprehensively.
In 7 years, 10 pediatric patients (9 liver transplants, 1 kidney transplant) were diagnosed with posttransplant lymphoproliferative disorder, with an incidence of 8.7% for pediatric liver transplants. Mean age at diagnosis was 46.4 months, and mean time from transplant to diagnosis was 21.2 months. The most common complaints at diagnosis included fever, lymphadenopathy, hepatosplenomegaly, dyspnea, and diarrhea. Treatment modalities included rituximab, immunosuppression reduction, intravenous immunoglobulin therapy, and chemotherapy (NHL Berlin-Frankfurt-Münster 90 protocols). All patients achieved remission (mean follow-up, 22.9 mo).
Early diagnosis of posttransplant lymphoproliferative disorder is important, and rituximab with immunosuppression reduction is effective to achieve complete remission, particularly in early polymorphic cases. Despite challenges, all patients achieved remission, signaling improved outcomes in pediatric posttransplant lymphoproliferative disorder. Active monitoring of Epstein?Barr virus infection may further reduce posttransplant lymphoproliferative disorder complications in pediatric solid-organ transplant; hence, early diagnosis is crucial.
移植后淋巴组织增生性疾病是实体器官移植后的一种严重并发症，特别是与 Epstein-Barr 病毒相关。我们研究了一个中心 7 年期间的儿科移植后淋巴组织增生性疾病病例，重点关注发病率、受累的解剖部位以及与临床结果的相关性。我们探讨了儿科移植后淋巴组织增生性疾病患者的临床特征和治疗结果，重点关注患者的生存和相关的临床后果。
这是对 2017 年 1 月 1 日至 2024 年 1 月 1 日期间在安卡拉 Baskent 大学器官移植中心接受儿科实体器官移植（肝或肾）的患者的病历进行的回顾性分析，该研究得到了机构审查委员会（KA24/63）的批准。我们根据病理证实的持续性淋巴结病或肿瘤性病变确定病例。根据肿瘤是恶性还是良性对患者进行分类。将早期移植后淋巴组织增生性疾病定义为移植后 1 年内。通过原位杂交确定 Epstein-Barr 病毒的相关性，并全面回顾患者特征。
在 7 年中，10 名儿科患者（9 例肝移植，1 例肾移植）被诊断为移植后淋巴组织增生性疾病，儿童肝移植的发病率为 8.7%。诊断时的平均年龄为 46.4 个月，从移植到诊断的平均时间为 21.2 个月。诊断时最常见的症状包括发热、淋巴结病、肝脾肿大、呼吸困难和腹泻。治疗方法包括利妥昔单抗、免疫抑制药物减少、静脉注射免疫球蛋白治疗和化疗（NHL Berlin-Frankfurt-Münster 90 方案）。所有患者均达到缓解（平均随访 22.9 个月）。
早期诊断移植后淋巴组织增生性疾病很重要，利妥昔单抗联合免疫抑制药物减少治疗对于实现完全缓解，特别是在早期多形性病例中是有效的。尽管存在挑战，但所有患者均达到缓解，表明儿科移植后淋巴组织增生性疾病的预后有所改善。积极监测 Epstein-Barr 病毒感染可能会进一步降低儿科实体器官移植后淋巴组织增生性疾病的并发症，因此早期诊断至关重要。
