Department of Surgery, Boston Children's Hospital, Boston, Massachusetts, USA.
Department of Nephrology and Hypertension, Rabin Medical Center, Petah Tikva, Israel.
Pediatr Transplant. 2022 Aug;26(5):e14268. doi: 10.1111/petr.14268. Epub 2022 Mar 19.
This study aimed to characterize features present at the time of diagnosis and describe outcomes in patients with post-transplant lymphoproliferative disorder (PTLD) following pediatric solid organ transplantation.
We performed a retrospective review of solid organ transplant patients who developed pathologically confirmed PTLD at our center from 2006 to 2016.
Of 594 patients included in this study, 41(6.9%) were diagnosed with PTLD. Median age at transplant was 5.6(IQR 1.7-16.1) years. Proportion of PTLD cases by organ transplanted and median time (IQR) to disease onset were: heart 11/144(7.6%) at 13.6(8.5-55.6) months, lung 7/52(13.5%) at 9.1(4.9-35) months, kidney 8/255(3.1%) at 39.5(13.9-57.1) months, liver 12/125(9.6%) at 7.7(5.5-22) months, intestine 0/4(0%), and multi-visceral 3/14(21.4%) at 5.4(5.4-5.6) months. No significant correlation was seen between recipient EBV status at transplant and timing of development of PTLD. There were six early lesions, 15 polymorphic, 19 monomorphic, and one uncharacterizable PTLD. Following immunosuppression reduction, 30 patients received rituximab, and 14 required chemotherapy. At median 25(IQR 12-53) months follow-up from the onset of PTLD, eight patients died secondary to transplant related complications, three are alive with active disease, and 30 have no evidence of disease.
PTLD is a significant complication following pediatric solid organ transplantation. EBV levels in conjunction with symptomatic presentation following transplant may assist in detection of PTLD. Most patients can achieve long-term disease-free survival through immunosuppression reduction, anti-CD20 treatment, and chemotherapy in refractory cases.
本研究旨在描述儿童实体器官移植后发生移植后淋巴组织增生性疾病（PTLD）患者的诊断时特征，并描述其结局。
我们对 2006 年至 2016 年在本中心经病理证实为 PTLD 的实体器官移植患者进行了回顾性研究。
本研究共纳入 594 例患者，其中 41 例（6.9%）诊断为 PTLD。移植时的中位年龄为 5.6（IQR 1.7-16.1）岁。按移植器官和发病时间（IQR）划分的 PTLD 病例比例分别为：心脏 144 例中的 11 例（7.6%），发病时间为 13.6（8.5-55.6）个月；肺 52 例中的 7 例（13.5%），发病时间为 9.1（4.9-35）个月；肾 255 例中的 8 例（3.1%），发病时间为 39.5（13.9-57.1）个月；肝 125 例中的 12 例（9.6%），发病时间为 7.7（5.5-22）个月；肠 4 例中无（0%），多器官 14 例中的 3 例（21.4%），发病时间为 5.4（5.4-5.6）个月。移植时受者 EBV 状态与 PTLD 发病时间无显著相关性。6 例为早期病变，15 例为多形性，19 例为单形性，1 例无法分类。在 PTLD 发病后，30 例患者接受了利妥昔单抗治疗，14 例患者需要化疗。PTLD 发病后中位随访 25（IQR 12-53）个月时，8 例患者因移植相关并发症死亡，3 例患者存活且疾病活动，30 例患者无疾病证据。
PTLD 是儿童实体器官移植后的严重并发症。移植后 EBV 水平结合症状表现可能有助于检测 PTLD。大多数患者可通过减少免疫抑制、抗 CD20 治疗和难治性病例的化疗实现长期无病生存。
