Devine Kaitlin, Ranganathan Sarangarajan, Mazariegos George, Bond Geoffrey, Soltys Kyle, Ganoza Armando, Sun Qing, Sindhi Rakesh
Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Pathology Department, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Pediatr Transplant. 2020 Aug;24(5):e13723. doi: 10.1111/petr.13723. Epub 2020 May 19.
Pediatric recipients of intestinal transplants have a high incidence of PTLD, but the impact of specific induction immunosuppression agents is unclear. In this single-center retrospective review from 2000 to 2017, we describe the incidence, characteristics, and outcomes of PTLD after primary intestinal transplantation in 173 children with or without liver, after induction with rATG, alemtuzumab, or anti-IL-2R agents. Thirty cases of PTLD occurred among 28 children, 28 EBV+ and 2 EBV-. Although not statistically significant, the PTLD incidence was higher after isolated intestinal transplant compared with liver-inclusive allograft (19.3% vs 13.3%, P = .393) and after induction with anti-IL-2R antibody and alemtuzumab compared with rATG (28.6% and 27.3% vs 13.3%, P = .076). The 30 PTLD cases included 13 monomorphic PTLD, 13 polymorphic PTLD, one spindle cell, one Burkitt lymphoma, and two cases too necrotic to classify. After reduction of immunosuppression, management was based on disease histology and extent. Resection with or without rituximab was used for polymorphic tumors and limited disease extent, whereas chemotherapy was used for diffuse disease. Of the 28 patients, 11 recovered with functioning allografts (39.3%), 10 recovered after enterectomy (35.7%), and seven patients died (25%), three due to PTLD and four due to other causes. All who died of progressive PTLD had received chemotherapy, highlighting the mortality of PTLD, toxicity of treatment and need for novel agents. Alemtuzumab is no longer used for induction at our center.
小儿肠移植受者发生移植后淋巴组织增生性疾病(PTLD)的几率很高,但特定诱导免疫抑制剂的影响尚不清楚。在这项对2000年至2017年的单中心回顾性研究中,我们描述了173例接受或未接受肝脏移植的儿童在接受兔抗胸腺细胞球蛋白(rATG)、阿仑单抗或抗白细胞介素-2受体(IL-2R)药物诱导后,初次肠移植后PTLD的发生率、特征和结局。28例儿童发生了30例PTLD,其中28例为EB病毒阳性(EBV+),2例为EB病毒阴性(EBV-)。虽然无统计学意义,但与包含肝脏的同种异体移植相比,单纯肠移植后PTLD的发生率更高(19.3%对13.3%,P = 0.393);与rATG相比,抗IL-2R抗体和阿仑单抗诱导后PTLD的发生率更高(28.6%和27.3%对13.3%,P = 0.076)。30例PTLD病例包括13例单形性PTLD、13例多形性PTLD、1例梭形细胞、1例伯基特淋巴瘤,还有2例因坏死严重无法分类。在免疫抑制降低后,治疗根据疾病组织学和范围进行。多形性肿瘤且疾病范围有限的患者采用手术切除加或不加利妥昔单抗治疗,而弥漫性疾病患者采用化疗。28例患者中,11例移植肠功能恢复(39.3%),10例肠切除后恢复(35.7%),7例死亡(25%),3例死于PTLD,4例死于其他原因。所有死于进行性PTLD的患者均接受了化疗,突出了PTLD的死亡率、治疗毒性以及对新型药物的需求。阿仑单抗在我们中心已不再用于诱导治疗。