Matsumura Muneyuki, Miyagi Shigehito, Tokodai Kazuaki, Kashiwadate Toshiaki, Fujio Atsushi, Miyazawa Koji, Sasaki Kengo, Saito Yoshikatsu, Kanai Norifumi, Unno Michiaki, Kamei Takashi
Department of Surgery Tohoku University Graduate School of Medicine Sendai Japan.
Clin Case Rep. 2022 Nov 4;10(11):e6454. doi: 10.1002/ccr3.6454. eCollection 2022 Nov.
Posttransplant lymphoproliferative disorder (PTLD) is a complication of solid organ transplantation and is associated with Epstein-Barr virus (EBV). Recently, EBV-related PTLD was defined as probable PTLD or proven PTLD. Probable PTLD involves significant lymphadenopathy, hepatosplenomegaly, or other end-organ manifestations, without a histological diagnosis, together with significant EBV DNAemia. Proven PTLD is the detection of EBV-encoded proteins in a tissue specimen, together with symptoms and/or signs originating from the affected organ. Probable PTLD after pediatric liver transplantation has not been well documented. Therefore, here, we aimed to describe cases of five pediatric patients with probable PTLD after liver transplantation, who were successfully treated with preemptive immunosuppression reduction with or without rituximab. All five patients (age range, 1-4 years; two girls and three boys) had EBV DNAemia. Three patients developed probable PTLD within 12 months of transplantation. Further, three patients had a significantly high EBV viral load, but the other two patients with lymphadenopathy and end-organ manifestation had a relatively low EBV viral load. Early onset pediatric PTLD with significant EBV DNAemia is almost universally EBV-related. Biopsy was not performed in any patient due to the relative inaccessibility of the lesion and young age of the patients. If the patient's symptoms are too mild, if excisional biopsy is too difficult to perform, or if the patient is too sick to undergo an invasive procedure, initiating preemptive treatment without a histological diagnosis could be the treatment option.
移植后淋巴细胞增生性疾病（PTLD）是实体器官移植的一种并发症，与 Epstein-Barr 病毒（EBV）相关。最近，EBV 相关的 PTLD 被定义为可能的 PTLD 或确诊的 PTLD。可能的 PTLD 包括显著的淋巴结病、肝脾肿大或其他终末器官表现，在没有组织学诊断的情况下，同时伴有显著的 EBV 血症。确诊的 PTLD 是在组织标本中检测到 EBV 编码蛋白，同时伴有源自受累器官的症状和/或体征。小儿肝移植后可能的 PTLD 尚未有充分的文献记载。因此，在此我们旨在描述 5 例小儿肝移植后可能患有 PTLD 的患者，他们通过减少免疫抑制治疗（无论是否使用利妥昔单抗）获得了成功治疗。所有 5 例患者（年龄范围 1 - 4 岁；2 名女孩和 3 名男孩）均有 EBV 血症。3 例患者在移植后 12 个月内出现可能的 PTLD。此外，3 例患者的 EBV 病毒载量显著升高，但另外 2 例有淋巴结病和终末器官表现的患者 EBV 病毒载量相对较低。伴有显著 EBV 血症的小儿早期 PTLD 几乎均与 EBV 相关。由于病变相对难以触及且患者年龄较小，所有患者均未进行活检。如果患者症状过于轻微，如果切除活检难以进行，或者如果患者病情过重无法接受侵入性操作，那么在没有组织学诊断的情况下启动抢先治疗可能是一种治疗选择。
