Ali Salah, AlThubaiti Sami, Renzi Samuele, Krueger Joerg, Chiang K Y, Naqvi Ahmed, Schechter Tal, Punnett Angela, Ali Muhammad
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
University of Toronto, Toronto, Ontario, Canada.
Pediatr Transplant. 2019 Feb;23(1):e13319. doi: 10.1111/petr.13319. Epub 2018 Nov 11.
EBV-related PTLD developing after HSCT is a potentially life-threatening disease. HLH is uncommon after allogeneic HSCT. Data on outcome of patients with PTLD and concomitant HLH after allogeneic HSCT are limited. In this retrospective study, we collected demographic, clinical, laboratory, and outcome data for 408 patients who underwent allogeneic HSCT from 2006 to 2015. Graft source included CB (n = 135; 33.1%), PBSCs (n = 34; 8.3%), and BM (n = 239; 58.6%). Eight out of 408 patients (2%) developed EBV-PTLD with a median age at HSCT of 5.9 years (range: 2.3-17.3). All eight patients received ATG as part of the conditioning regimen. Graft source was PBSC in three patients (37.5%), BM in four patients (50%), and CB in one patient (12.5%). Donors were matched unrelated in five patients (62.5%) and matched sibling in three patients (37.5%). Seven out of eight patients developed EBV-PTLD within the first 100-day post-HSCT. Lymph node biopsy revealed early lesions in three patients, polymorphic in three patients, and monomorphic PTLD in two patients. Three patients (37.5%) died within 1 month of EBV-PTLD diagnosis. All deceased patients developed HLH manifestations with two of them meeting HLH diagnostic criteria and one having an incomplete workup. PTLD after allogeneic HSCT with manifestations of HLH is associated with high mortality. Early identification and treatment of EBV-PTLD seems imperative to control the disease, especially if signs of HLH are evolving.
造血干细胞移植（HSCT）后发生的与EB病毒（EBV）相关的移植后淋巴增殖性疾病（PTLD）是一种潜在的危及生命的疾病。噬血细胞性淋巴组织细胞增生症（HLH）在异基因HSCT后并不常见。关于异基因HSCT后PTLD合并HLH患者的预后数据有限。在这项回顾性研究中，我们收集了2006年至2015年接受异基因HSCT的408例患者的人口统计学、临床、实验室和预后数据。移植物来源包括脐血（CB，n = 135；33.1%）、外周血干细胞（PBSCs，n = 34；8.3%）和骨髓（BM，n = 239；58.6%）。408例患者中有8例（2%）发生EBV-PTLD，HSCT时的中位年龄为5.9岁（范围：2.3 - 17.3岁）。所有8例患者均接受抗胸腺细胞球蛋白（ATG）作为预处理方案的一部分。3例患者（37.5%）的移植物来源为PBSC，4例患者（50%）为BM，1例患者（12.5%）为CB。5例患者（62.5%）的供者为匹配的无关供者，3例患者（37.5%）为匹配的同胞供者。8例患者中有7例在HSCT后的前100天内发生EBV-PTLD。淋巴结活检显示3例患者为早期病变，3例为多形性病变，2例为单形性PTLD。3例患者（37.5%）在EBV-PTLD诊断后1个月内死亡。所有死亡患者均出现HLH表现，其中2例符合HLH诊断标准，1例检查不完整。异基因HSCT后出现HLH表现的PTLD与高死亡率相关。早期识别和治疗EBV-PTLD对于控制疾病似乎至关重要，尤其是当HLH迹象正在发展时。
