Department of Pediatric Hematology-Oncology, Ospedale Bambino Gesù, Rome, Italy.
Pediatrics. 2012 Jan;129(1):e189-94. doi: 10.1542/peds.2011-0636. Epub 2011 Dec 5.
Epstein-Barr virus (EBV)-related post-transplantation lymphoproliferative disorder (PTLD) is a life-threatening complication in patients given T-cell-depleted hematopoietic stem cell transplantation from an HLA-haploidentical relative (haplo-HSCT). We report the case of a child who developed severe EBV-related PTLD after haplo-HSCT from his mother. Despite receiving the anti-CD20 monoclonal antibody, the patient presented with intestinal obstruction due to huge abdominal lymphadenopathy, hematemesis, and nodulary pulmonary lesions. Histology showed that the lesions were due to CD20-/CD19+ large neoplastic B cells. The patient underwent double intestinal resection with partial abdominal lymphadenectomy and then received 3 monthly doses of donor-derived unmanipulated mononuclear cells. The initial dose of CD3+ cells was 3 10(5)/kg recipient body weight. The 2 additional doses consisted of 5 10(5) CD3+ cells/kg. No sign or symptom attributable to graft-versus-host disease was observed, and the patient completely cleared EBV-related lesions. The child was disease-free for 13 months after the first lymphocyte infusion. This case demonstrates that repeated infusions of controlled numbers of donor CD3+ cells cure EBV-related PTLD in haplo-HSCT without inducing graft-versus-host disease.
译文： 移植后淋巴细胞增生性疾病（PTLD）是 T 细胞耗竭的造血干细胞移植来自 HLA 单倍体相关供体（haplo-HSCT）的患者的危及生命的并发症。我们报告了一例患儿在接受来自母亲的haplo-HSCT 后发生严重 EBV 相关 PTLD 的病例。尽管接受了抗 CD20 单克隆抗体治疗，但该患者因巨大的腹部淋巴结病、呕血和结节性肺病变而出现肠梗阻。组织学显示病变是由 CD20-/CD19+大肿瘤性 B 细胞引起的。患者接受了双肠切除术和部分腹部淋巴结切除术，然后接受了 3 个月剂量的供体来源未处理的单核细胞。初始剂量为 3×10(5)/kg 受者体重的 CD3+细胞。另外 2 剂包含 5×10(5) CD3+细胞/kg。未观察到移植物抗宿主病的迹象或症状，患者完全清除了 EBV 相关病变。第一次淋巴细胞输注后，患儿无病生存 13 个月。该病例表明，重复输注受控数量的供体 CD3+细胞可在不引起移植物抗宿主病的情况下治愈 haplo-HSCT 中的 EBV 相关 PTLD。
