Gong Jerald Z, Bayerl Michael G, Sandhaus Linda M, Sebastian Siby, Rehder Catherine W, Routbort Mark, Lagoo Anand S, Szabolcs Paul, Chiu Jeanie, Comito Melanie, Buckley Patrick J
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Am J Surg Pathol. 2006 Mar;30(3):328-36. doi: 10.1097/01.pas.0000188030.63706.e7.
Reported are 7 cases of posttransplant lymphoproliferative disorder (PTLD) arising in children who received umbilical cord blood transplantation (UCBT). There were 4 females and 3 males with a median age of 3 years (range, 1-16 years). All 7 patients received UCBT, including 1 patient who received multiple units and 1 transplanted under nonmyeloablative condition. The time interval from UCBT to PTLD averaged 4 months (range, 2 weeks to 9 months). Patients typically presented with high-stage disease with visceral organ involvement. Histology of the PTLDs showed monomorphic morphology in 5 cases and polymorphic morphology in the remaining 2 cases. Bone marrow biopsies were performed in 3 cases and were negative for PTLD. Epstein-Barr virus (EBV) was detected in the PTLD in all 7 patients by in situ hybridization. Evidence of past EBV infection was found in the recipients, but the EBV genome was not detected in the donor cord blood samples, suggesting that donor cord blood was not the source of EBV infection. The origin of the PTLD was investigated in 5 cases. PTLD was of host origin in 2 patients who failed engraftment and of donor origin in the remaining 3 patients who had complete engraftment. Four of 5 patients with monomorphic PTLD failed to demonstrate significant responses to rituximab and/or reduction of immunosuppression and died within 1 month after diagnosis. The remaining 2 patients with polymorphic PTLD showed complete response to therapy. One patient was alive 35 months after transplant, and the other patient died of infection 6 months after transplant. It is concluded that PTLD arising after UCBT in children occurs early after transplant and represents a serious EBV-related complication. PTLD may be of donor or recipient origin depending on engraftment status. Both monomorphic and polymorphic histology may be seen, and monomorphic histology appears to predict an unfavorable prognosis.
报告了7例接受脐带血移植（UCBT）的儿童发生的移植后淋巴细胞增生性疾病（PTLD）。其中有4名女性和3名男性，中位年龄为3岁（范围1 - 16岁）。所有7例患者均接受了UCBT，包括1例接受多单位移植的患者和1例在非清髓条件下移植的患者。从UCBT到PTLD的时间间隔平均为4个月（范围2周至9个月）。患者通常表现为累及内脏器官的晚期疾病。PTLD的组织学检查显示，5例为单形性形态，其余2例为多形性形态。3例进行了骨髓活检，结果显示PTLD为阴性。通过原位杂交在所有7例患者的PTLD中均检测到爱泼斯坦 - 巴尔病毒（EBV）。在受者中发现了既往EBV感染的证据，但在供体脐带血样本中未检测到EBV基因组，提示供体脐带血不是EBV感染的来源。对5例患者的PTLD起源进行了研究。在2例植入失败的患者中，PTLD起源于宿主，在其余3例完全植入的患者中，PTLD起源于供体。5例单形性PTLD患者中有4例对利妥昔单抗和/或免疫抑制的降低未表现出显著反应，并在诊断后1个月内死亡。其余2例多形性PTLD患者对治疗表现出完全反应。1例患者在移植后35个月存活，另1例患者在移植后6个月死于感染。结论是，儿童UCBT后发生的PTLD在移植后早期出现，是一种严重的EBV相关并发症。PTLD可能根据植入状态起源于供体或受体。单形性和多形性组织学均可出现，单形性组织学似乎预示预后不良。
