[相关hla -单倍体外周血干细胞移植3年后发生进行性多灶性白质脑病]。

Kumiko Ando, Kazuo Nakamichi, Kiyoharu Hirose, Yuichi Taneyama, Harumi Kakuda, Hidemasa Ochiai
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引用次数: 0

摘要

患者是一名22岁的男性。8年前,他患上t细胞淋巴母细胞淋巴瘤,在治疗期间复发。他接受了来自相关供者的hla -单倍体外周血干细胞移植(PBSCT)并获得缓解。移植后,他出现了严重的慢性移植物抗宿主病(GVHD),全身受累,需要长期服用几种免疫抑制药物。移植后大约3年零9个月,他出现了抑郁、厌食症和体重下降的症状。脑部MRI示t2wi高信号病变,由小脑右半球延伸至小脑中脚,轻度强化,怀疑淋巴瘤复发。最后,实时荧光定量PCR检测脑脊液(CSF)中JC病毒(JCV)为61拷贝/ml阳性,诊断为进行性多灶性脑白质病(PML)。我们认为患者移植后继发性免疫缺陷延长,并逐渐减少其免疫抑制药物。此后,脑脊液中的JCV对检测的敏感性降低。该病例强调了监测PML作为造血干细胞移植后潜在的晚期并发症的重要性,并提供了有价值的见解,即只有通过减少剂量和停用免疫抑制药物才能实现改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Progressive multifocal leukoencephalopathy developed 3 years after related HLA-haploidentical peripheral blood stem cell transplantation].

The patient was a 22-year-old man. 8 years ago, he developed T-cell lymphoblastic lymphoma and relapsed during treatment. He underwent HLA-haploidentical peripheral blood stem cell transplantation (PBSCT) from a related donor and achieved remission. After transplantation, he developed severe chronic graft-versus-host disease (GVHD) with systemic involvement, requiring long-term administration of several immunosuppressive drugs. About 3 years and 9 months after the transplant, he was experiencing depression, anorexia, and weight loss. Brain MRI showed hyperintense lesions in T2-weighted imaging extending from the right cerebellar hemisphere to the middle cerebellar peduncle with slight enhancement, and lymphoma recurrence was also suspected. Finally, a quantitative real-time PCR test was positive for JC virus (JCV) in the cerebrospinal fluid (CSF) at 61 copies/ml, leading to a diagnosis of progressive multifocal leukoencephalopathy (PML). We considered the patient to have prolonged secondary immunodeficiency after transplantation, and gradually reduced his immunosuppressive drugs. After that, the JCV in CSF became less sensitive to detection. This case highlights the importance of monitoring for PML as a potential late complication after hematopoietic stem cell transplantation, and provides the valuable insight that improvement was achieved only through dose reduction and discontinuation of immunosuppressive drugs.

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