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进行性多灶性白质脑病:3例HIV阴性血液病患者报告及文献复习

Progressive multifocal leukoencephalopathy: report of three cases in HIV-negative hematological patients and review of literature.

作者信息

Pelosini Matteo, Focosi Daniele, Rita Fazzi, Galimberti Sara, Caracciolo Francesco, Benedetti Edoardo, Papineschi Federico, Petrini Mario

机构信息

Division of Haematology, Department of Oncology, Transplants and Advances in Medicine, University of Pisa, Pisa, Italy.

出版信息

Ann Hematol. 2008 May;87(5):405-12. doi: 10.1007/s00277-007-0411-6. Epub 2007 Dec 7.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a central nervous system (CNS) disease usually observed in immunodeficient patients, especially human immunodeficiency virus (HIV)-positive, caused by John Cunningham virus. This infectious complication has been described in many HIV-negative hematological patients, especially affected by lymphoproliferative diseases. PML has been observed after both chemotherapy and bone marrow transplantation and, recently, in association with rituximab. Diagnosis can be complicated, and often a CNS biopsy is required. Current treatment approaches are not effective in both HIV-positive and HIV-negative patients, and the outcome remain very poor in the majority of cases, even after combination therapies. We report three cases of PML in hematological patients, treated respectively with conventional chemotherapy and autologous and haploidentical transplantation, and review the literature on PML. All of them received rituximab, which has recently been in the focus of a Food and Drug Administration warning.

摘要

进行性多灶性白质脑病(PML)是一种中枢神经系统(CNS)疾病,通常见于免疫缺陷患者,尤其是人类免疫缺陷病毒(HIV)阳性患者,由约翰·坎宁安病毒引起。这种感染性并发症在许多HIV阴性血液系统疾病患者中也有描述,尤其是受淋巴增殖性疾病影响的患者。PML在化疗和骨髓移植后均有观察到,最近还与利妥昔单抗有关。诊断可能很复杂,通常需要进行中枢神经系统活检。目前的治疗方法对HIV阳性和HIV阴性患者均无效,即使采用联合治疗,大多数病例的预后仍然很差。我们报告了3例血液系统疾病患者的PML病例,分别接受了传统化疗、自体移植和单倍体移植,并对PML的相关文献进行了综述。他们都接受了利妥昔单抗治疗,而利妥昔单抗最近已成为美国食品药品监督管理局警告的焦点。

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