Awaya Norihiro, Adachi Akiko, Mori Taisuke, Kamata Hiroshi, Nakahara Jin, Yokoyama Kenji, Yamada Taketo, Kizaki Masahiro, Sakamoto Michiie, Ikeda Yasuo, Okamoto Shin-ichiro
Department of Medicine, Division of Hematology, Keio University School of Medicine, Japan.
Leuk Res. 2006 Aug;30(8):1059-62. doi: 10.1016/j.leukres.2005.10.022. Epub 2005 Dec 2.
Post-transplant lymphoproliferative disorder (PTLD) is a complication that can develop after either solid-organ or hematopoietic stem cell transplantation (HSCT). T-cell PTLD is a rare disorder, especially following autologous HSCT. Here we report a case of T-cell PTLD which occurred after autologous peripheral blood stem cell transplantation (PBSCT) for relapsed angioimmunoblastic T-cell lymphoma (AILT). Three months after the transplant, the patient developed fever with elevated plasma Epstein-Barr virus (EBV)-PCR values. The patient subsequently developed pneumonitis, hepatomegaly and marked pancytopenia due to hemophagocytosis. The patient died of multi-organ failure, despite antiviral and steroid pulse therapy. Our post-mortem study confirmed the marked proliferation of EBV-infected T-cells that differed from the original AILT clone and macrophages/histiocytes were observed in the marrow, liver, lymph nodes and lungs. Phagocytosis was most evident in the bone marrow. The patient's AILT remained in complete remission. To the best of our knowledge, this is the first case of fulminant EBV-associated T-cell lymphoproliferative disorder (LPD) following autologous HSCT.
移植后淋巴细胞增生性疾病（PTLD）是一种可在实体器官移植或造血干细胞移植（HSCT）后发生的并发症。T细胞PTLD是一种罕见疾病，尤其是在自体HSCT后。在此，我们报告1例T细胞PTLD病例，该病例发生在自体外周血干细胞移植（PBSCT）治疗复发性血管免疫母细胞性T细胞淋巴瘤（AILT）之后。移植后3个月，患者出现发热，血浆EB病毒（EBV）-PCR值升高。患者随后因噬血细胞作用出现肺炎、肝肿大和明显的全血细胞减少。尽管进行了抗病毒和类固醇冲击治疗，患者仍死于多器官功能衰竭。我们的尸检研究证实了EBV感染的T细胞显著增殖，其不同于原始的AILT克隆，并且在骨髓、肝脏、淋巴结和肺中观察到巨噬细胞/组织细胞。吞噬作用在骨髓中最为明显。患者的AILT仍处于完全缓解状态。据我们所知，这是自体HSCT后暴发性EBV相关T细胞淋巴增生性疾病（LPD）的首例病例。
