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免疫系统在进行性多灶性白质脑病中的作用:自体和异基因造血干细胞移植后两例病例的对比分析

The role of the immune system in progressive multifocal leukoencephalopathy: a comparative analysis of two cases following autologous and allogeneic hematopoietic stem cell transplantation.

作者信息

Mandelli Biancamaria, Mazzarella Roberta, Corbingi Andrea, Barbera Elettra Ortu La, Perrone Salvatore, Fanciullo David, Lorenzon Martina, Pacitto Giada, Cenfra Natalia, Mecarocci Sergio, Asioli Sofia, Pulsoni Alessandro

机构信息

Hematology, Department of Translational and Precision Medicine, Az. Policlinico Umberto I-Sapienza University, Rome, Italy.

Department of Hematology, Polo Universitario Pontino, S.M. Goretti Hospital, Latina, Italy.

出版信息

Ann Hematol. 2025 Jun;104(6):3505-3510. doi: 10.1007/s00277-025-06258-5. Epub 2025 May 5.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a rare, subacute demyelinating disorder of the central nervous system (CNS) caused by the JCV. In immunosuppressed hosts, PML is caused by reactivation of a latent infection rather than primary exposure. Hematological patients, particularly post-transplant, presenting with worsening neurological symptoms should promptly consider PML in the differential diagnosis. The rarity of PML after autologous or allogeneic hematopoietic stem cell transplantation (HSCT) and the absence of a universally effective therapy represents a clinical challenge. Here, we present two cases of PML developed after autologous and allogeneic HSCT, with completely different outcomes dependent on the patients' clinical backgrounds and the level of immune system competence which is the key factor in determining either the onset or viral clearance of the infection.

摘要

进行性多灶性白质脑病(PML)是一种由JC病毒引起的罕见的中枢神经系统(CNS)亚急性脱髓鞘疾病。在免疫抑制宿主中,PML是由潜伏感染的重新激活而非初次暴露引起的。血液系统疾病患者,尤其是移植后患者,若出现进行性神经症状,在鉴别诊断时应及时考虑PML。自体或异基因造血干细胞移植(HSCT)后发生PML的情况罕见,且缺乏普遍有效的治疗方法,这是一个临床挑战。在此,我们报告两例自体和异基因HSCT后发生PML的病例,其结局完全不同,这取决于患者的临床背景和免疫系统功能水平,而免疫系统功能水平是决定感染发作或病毒清除的关键因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18d2/12283870/e2f5871dbe16/277_2025_6258_Fig1_HTML.jpg

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