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血液系统恶性肿瘤和干细胞移植后进行性多灶性白质脑病的特征和结局:病例系列研究。

Characteristics and outcomes of progressive multifocal leukoencephalopathy in hematologic malignancies and stem cell transplant - a case series.

机构信息

a Department of Medical Oncology, Albert Einstein College of Medicine/Montefiore Medical Center , Bronx , NY , USA.

b Department of Medicine, Division of Hematology , Ohio State University , Columbus , OH , USA.

出版信息

Leuk Lymphoma. 2019 Feb;60(2):395-401. doi: 10.1080/10428194.2018.1474523. Epub 2018 Jul 3.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a life-threatening opportunistic infection of immunomodulatory therapies. PML cases reported in PubMed (1995-2017) following stem-cell transplantation (HSCT) or chemoimmunotherapy (CIT) for hematologic malignancies were reviewed. We found 107 cases, 40% were HSCT recipients (32 allogeneic, 11 autologous) and 40% indolent lymphomas receiving monoclonal antibodies (mAbs). HSCT cases had longer time to PML diagnosis (10.8 vs. 4 months, p < .001), higher proportion of PML therapy response (58% vs. 25%, p = .019), lower mortality rate (56% vs. 88%, p < .001), and longer median survival (8 vs. 2 months, p < .001). Outcome differences might be caused by selection bias as HSCT patients are most likely treated aggressively; however, time-dependent immune reconstitution might also contribute to their better prognosis. Increased use of mAbs and HSCT are associated with rising PML incidence in hematological malignancies, currently constituting the second largest vulnerable population after HIV-infected patients; further research is needed for its optimal treatment.

摘要

进行性多灶性白质脑病(PML)是一种危及生命的机会性感染,发生于免疫调节治疗后。对 PubMed 中(1995-2017 年)报道的造血系统恶性肿瘤患者接受造血干细胞移植(HSCT)或化疗免疫治疗(CIT)后发生的 PML 病例进行了回顾。共发现 107 例患者,40%为 HSCT 受者(32 例异基因,11 例自体),40%为惰性淋巴瘤患者接受单克隆抗体(mAb)治疗。HSCT 患者的 PML 诊断时间更长(10.8 个月 vs. 4 个月,p<0.001),PML 治疗反应比例更高(58% vs. 25%,p=0.019),死亡率更低(56% vs. 88%,p<0.001),中位生存期更长(8 个月 vs. 2 个月,p<0.001)。预后差异可能是由于选择偏倚所致,因为 HSCT 患者最有可能接受积极治疗;然而,随着时间的推移免疫重建也可能导致其预后较好。mAb 和 HSCT 的应用增加与血液系统恶性肿瘤中 PML 发病率的上升有关,目前仅次于 HIV 感染患者,是第二大易感人群;需要进一步研究以优化其治疗方法。

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