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

IF 2.4 3区 医学 Q2 HEMATOLOGY
Annals of Hematology Pub Date : 2025-06-01 Epub Date: 2025-05-05 DOI:10.1007/s00277-025-06258-5
Biancamaria Mandelli, Roberta Mazzarella, Andrea Corbingi, Elettra Ortu La Barbera, Salvatore Perrone, David Fanciullo, Martina Lorenzon, Giada Pacitto, Natalia Cenfra, Sergio Mecarocci, Sofia Asioli, Alessandro Pulsoni
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引用次数: 0

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.

免疫系统在进行性多灶性白质脑病中的作用:自体和异体造血干细胞移植后两例的比较分析。
进行性多灶性脑白质病(PML)是一种罕见的由JCV引起的中枢神经系统(CNS)亚急性脱髓鞘疾病。在免疫抑制的宿主中,PML是由潜伏感染的再激活而不是初次暴露引起的。血液学患者,特别是移植后出现神经系统症状恶化时,应及时考虑PML作为鉴别诊断的依据。自体或异体造血干细胞移植(HSCT)后PML的罕见性和缺乏普遍有效的治疗方法代表了临床挑战。在这里,我们报告了两例自体和异体造血干细胞移植后发生的PML,其结果完全不同,取决于患者的临床背景和免疫系统能力水平,而免疫系统能力水平是决定感染发生或病毒清除的关键因素。
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来源期刊
Annals of Hematology
Annals of Hematology 医学-血液学
CiteScore
5.60
自引率
2.90%
发文量
304
审稿时长
2 months
期刊介绍: Annals of Hematology covers the whole spectrum of clinical and experimental hematology, hemostaseology, blood transfusion, and related aspects of medical oncology, including diagnosis and treatment of leukemias, lymphatic neoplasias and solid tumors, and transplantation of hematopoietic stem cells. Coverage includes general aspects of oncology, molecular biology and immunology as pertinent to problems of human blood disease. The journal is associated with the German Society for Hematology and Medical Oncology, and the Austrian Society for Hematology and Oncology.
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