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病例报告:儿童肾移植受者发生 EB 病毒相关移植后淋巴增殖性疾病和噬血细胞性淋巴组织细胞增生症后的高炎症性毒性反应

英文原题:Case Report: Hyperinflammatory toxicities after Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and hemophagocytic lymphohistiocytosis in a pediatric kidney transplant recipient.

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Case Report: Hyperinflammatory toxicities after Epstein-Barr virus-associated post-transplant lymphoproliferative disorder and hemophagocytic lymphohistiocytosis in a pediatric kidney transplant recipient.

PubMed 2026/06/10(内容时间) Front Immunol Q1 · IF 7(JCR 2025)

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研究概要

本病例凸显了实体器官移植受者中 EBV-HLH 的治疗复杂性,并强调了在严重高炎症反应和器官功能障碍情况下细胞免疫治疗的理论依据与风险。

研究思路结论见上方概要

EBV 是继发性 HLH 的常见诱因,尤其是在移植受者中。使用 EBV 特异性 T 细胞的过继性细胞治疗是难治性 EBV 驱动疾病的一种新兴选择,但可能诱发细胞因子毒性。病例:我们报告一名有先天性肾病综合征病史的男孩,其在婴儿期接受了母体肾移植。6 岁时,他发生了单形性 EBV 阳性移植后淋巴增殖性疾病,随后出现 EBV 驱动的 HLH。尽管接受了依托泊苷和类固醇为基础的 HLH 治疗、IVIG、anakinra 以及母体病毒特异性 T 细胞输注,他仍表现出复发性过度炎症。在单次给予异体 EBV 特异性 T 细胞产品 tabelecleucel(Ebvallo)且 HLH 加重后,他出现了细胞因子释放综合征和神经毒性,并伴有急性肝衰竭、凝血病和肾衰竭。他死于难治性乳酸酸中毒和多器官衰竭。

展开英文摘要原文

Epstein-Barr virus (EBV) is a common trigger of secondary hemophagocytic lymphohistiocytosis (HLH), particularly in transplant recipients. Adoptive cellular therapy with EBV-specific T cells is an emerging option for refractory EBV-driven disease but may precipitate cytokine toxicities. CASE: We report a boy with a history of congenital nephrotic syndrome who in infancy underwent maternal kidney transplantation. At the age of 6 years, he developed monomorphic EBV-positive post-transplant lymphoproliferative disorder, followed by EBV-driven HLH. Despite etoposide- and steroid-based HLH therapy, IVIG, anakinra, and a maternal virus-specific T-cell infusion, he showed recurrent hyperinflammation. After a single dose of the allogeneic EBV-specific T-cell product tabelecleucel (Ebvallo) and exacerbation of HLH, he experienced cytokine release syndrome and neurotoxicity with acute liver failure, coagulopathy, and renal failure. He died from refractory lactic acidosis and multiorgan failure.

This case highlights the therapeutic complexity of EBV-HLH in a solid-organ transplant recipient and underscores both the rationale for and risks of cellular immunotherapy amid severe hyperinflammation and organ dysfunction.

论文信息

作者
Ussowicz M、Jarmużek W、Rutynowska-Pronicka O、Śliwińska A、Bogacz A、Zielińska M、Litwin M、Dembowska-Bagińska B
第一作者单位
Department of Paediatric Bone Marrow Transplantation, Oncology, and Hematology, Wroclaw Medical University, Wrocław, Poland.Poland
通讯作者单位
Department of Oncology, The Children's Memorial Health Institute, Warsaw, Poland.Poland
文献类型
病例报告
期刊
Frontiers in immunology2026
原文标识
PubMed 42358947 · DOI 10.3389/fimmu.2026.1840732