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移植受者的进行性多灶性白质脑病

Progressive multifocal leukoencephalopathy in transplant recipients.

作者信息

Shitrit David, Lev Nirit, Bar-Gil-Shitrit Ariella, Kramer Mordechai R

机构信息

Pulmonary Institute, Rabin Medical Center, Beilinson Campus, 49100, Petah Tiqwa, Israel.

出版信息

Transpl Int. 2005 Jan;17(11):658-65. doi: 10.1007/s00147-004-0779-3. Epub 2004 Dec 23.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating infection caused by the JC virus. It is an emerging disease in transplant recipients; however, it remains poorly defined. Twenty-four cases of PML reported in the literature in transplant recipients were reviewed. Of the 24 cases, nine occurred in renal, six in bone marrow, four in liver, three in heart and two in lung transplant recipients. Median time to onset was 17 months; 71% occurred within 24 months of transplantation. PML tended to occur later in the kidney recipients (P=0.04). Seventy-five percent had subacute presentation; hemiparesis (50%), apathy (46%) and confusion (38%) were the most frequently presented features. Treatment included reduction of immunosuppression and chemotherapy, mainly cidofovir. Death occurred within 2.5 months of the onset of symptoms in 17 patients (71%). PML is a unique entity that should be considered in any transplant recipient with neurological symptoms. The outcome is usually fatal, although regression has been reported.

摘要

进行性多灶性白质脑病(PML)是一种由JC病毒引起的脱髓鞘感染性疾病。它是移植受者中一种新出现的疾病;然而,其定义仍不明确。对文献中报道的24例移植受者的PML病例进行了回顾。在这24例病例中,9例发生在肾移植受者中,6例在骨髓移植受者中,4例在肝移植受者中,3例在心脏移植受者中,2例在肺移植受者中。发病的中位时间为17个月;71%的病例在移植后24个月内发病。PML在肾移植受者中发病时间往往较晚(P=0.04)。75%的患者表现为亚急性;偏瘫(50%)、淡漠(46%)和意识模糊(38%)是最常见的表现。治疗包括减少免疫抑制和化疗,主要是西多福韦。17例患者(71%)在症状出现后2.5个月内死亡。PML是一种独特的疾病,任何有神经症状的移植受者都应考虑到该病。尽管有病情缓解的报道,但预后通常是致命的。

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