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首页> 外文期刊>Journal of neurology >Progressive multifocal leukoencephalopathy in patients treated with fumaric acid esters: a review of 19 cases
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Progressive multifocal leukoencephalopathy in patients treated with fumaric acid esters: a review of 19 cases

机译：富马酸酯治疗患者的渐进式多焦点白血病：综述19例

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摘要

Progressive multifocal leukoencephalopathy (PML) is a rare and potentially fatal condition caused by a brain infection with JC polyomavirus (JCV). PML develops almost exclusively in immunocompromised patients and has recently been associated with use of fumaric acid esters (FAEs), or fumarates. We reviewed the literature and the Dutch and European pharmacovigilance databases in order to identify all available FAE-associated PML cases and distinguish possible common features among these patients. A total of 19 PML cases associated with FAE use were identified. Five cases were associated with FAE use for multiple sclerosis and 14 for psoriasis. Ten patients were male and nine were female. The median age at PML diagnosis was 59 years. The median duration of FAE therapy to PML symptom onset or appearance of first PML lesion on brain imaging was 31 months (range 6-110). In all cases a certain degree of lymphocytopenia was reported. The median duration of lymphocytopenia to PML symptom onset was 23 months (range 6-72). The median lymphocyte count at PML diagnosis was 414 cells/mu L. CD4 and CD8 counts were reported in ten cases, with median cell count of 137 and 39 cells/mu L, respectively. Three patients died (16% mortality). The association between occurrence of PML in patients with low CD4 and CD8 counts is reminiscent of PML cases in the HIV population and suggests that loss of T cells is the most important risk factor.
机译：渐进式多焦点白血病（PML）是由JC PolyomaVirus（JCV）的脑感染引起的罕见和潜在的致命条件。 PML几乎完全在免疫疗效患者中开发，最近与富马酸酯（FAE）或富马酸盐有关。我们审查了文献和荷兰语和欧洲药物知识数据库，以识别所有可用的FAE相关的PML病例，并区分这些患者之间可能的共同特征。还确定了与FAE使用相关的19个PML病例。五种病例与FAE用于多发性硬化和14例用于牛皮癣有关。十名患者是男性，九是女性。 PML诊断的中位年龄为59年。 FAE治疗的中位持续时间对PML症状发作或脑成像上的第一个PML病变的出现为31个月（范围6-110）。在所有情况下，报告了一定程度的淋巴细胞症。淋巴细胞症的中值持续时间为PML症状发作为23个月（范围6-72）。 PML诊断的中位数淋巴细胞计数为414个细胞/ mu L. CD4和CD8计数在十个病例中报告，分别是137和39个细胞/μl的中值计数。三名患者死亡（16％死亡率）。低CD4和CD8计数患者的PML之间的关联是在艾滋病毒群体中让PML病例中兴起来，表明T细胞的损失是最重要的危险因素。

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