Progressive multifocal leukoencephalopathy in transplant recipients.
Progressive multifocal leukoencephalopathy in transplant recipients.
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DOI：
10.1002/ana.22408
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发表时间：
2011-08
影响因子：
11.2
通讯作者：
Nath A
中科院分区：
文献类型：
--
作者：
Mateen FJ;Muralidharan R;Carone M;van de Beek D;Harrison DM;Aksamit AJ;Gould MS;Clifford DB;Nath A
移植受者有患进行性多灶性白质脑病 (PML) 的风险，这是一种罕见的脱髓鞘疾病，由 JC 病毒破坏少突胶质细胞引起。使用 PubMed Entrez（1958 年至 2010 年 7 月）发现了移植后发生 PML 的报告。一项多中心、回顾性队列研究还确定了在梅奥诊所、约翰·霍普金斯大学、华盛顿大学和阿姆斯特丹学术医学中心诊断的移植受者中的所有 PML 病例。在 1 个机构计算了移植后 PML 的发生率。共发现移植后PML病例69例（44例实体器官，25例骨髓），其中15例来自4个医疗中心，另外54例来自文献。实体器官移植后出现 PML 首发症状的中位时间比骨髓受者更长（27 个月与 11 个月，p = 0.0005，范围 <1 至 >240）。实体器官受者症状出现后的中位生存期为 6.4 个月，而骨髓受者的中位生存期为 19.5 个月 (p = 0.068)。病死率为 84%（95% 置信区间 [CI]，70.3–92.4%），1 年以上生存率为 55.7%（95% CI，41.2–67.2%）。 1 个机构的心脏和/或肺移植受者中 PML 的发生率为每 1,000 名移植后人年 1.24 例（95% CI，0.25-3.61）。未发现与任何 1 种免疫抑制剂有明显关联。没有任何治疗提供明显的治疗效果。 PML 的风险存在于整个移植后时期。骨髓接受者的存活时间比实体器官接受者的存活时间更长，但出现 PML 首次症状的中位时间可能较短。移植后 PML 的病死率较高，并且可能比接受高效抗逆转录病毒治疗 (HAART) 的人类免疫缺陷病毒 (HIV) 患者或接受那他珠单抗治疗的多发性硬化症患者报告的发病率更高。
Transplant recipients are at risk of developing progressive multifocal leukoencephalopathy (PML), a rare demyelinating disorder caused by oligodendrocyte destruction by JC virus. Reports of PML following transplantation were found using PubMed Entrez (1958–July 2010). A multicenter, retrospective cohort study also identified all cases of PML among transplant recipients diagnosed at Mayo Clinic, Johns Hopkins University, Washington University, and Amsterdam Academic Medical Center. At 1 institution, the incidence of posttransplantation PML was calculated. A total of 69 cases (44 solid organ, 25 bone marrow) of posttransplantation PML were found including 15 from the 4 medical centers and another 54 from the literature. The median time to development of first symptoms of PML following transplantation was longer in solid organ vs bone marrow recipients (27 vs 11 months, p = 0.0005, range of <1 to >240). Median survival following symptom onset was 6.4 months in solid organ vs 19.5 months in bone marrow recipients (p = 0.068). Case fatality was 84% (95% confidence interval [CI], 70.3–92.4%) and survival beyond 1 year was 55.7% (95% CI, 41.2–67.2%). The incidence of PML among heart and/or lung transplant recipients at 1 institution was 1.24 per 1,000 posttransplantation person-years (95% CI, 0.25–3.61). No clear association was found with any 1 immunosuppressant agent. No treatment provided demonstrable therapeutic benefit. The risk of PML exists throughout the posttransplantation period. Bone marrow recipients survive longer than solid organ recipients but may have a lower median time to first symptoms of PML. Posttransplantation PML has a higher case fatality and may have a higher incidence than reported in human immunodeficiency virus (HIV) patients on highly-active antiretroviral therapy (HAART) or multiple sclerosis patients treated with natalizumab.