Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy

Predictive factors for prolonged survival in acquired immunodeficiency syndrome-associated progressive multifocal leukoencephalopathy
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DOI:
10.1002/ana.410440309
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发表时间:
1998-09-01
影响因子:
11.2
通讯作者:
Dobbs, M
Dobbs, M
中科院分区:
医学1区
文献类型:
--
作者:
Berger, JR;Levy, RM;Dobbs, M

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进行性多灶性白质脑病(PML)并发获得性免疫缺陷综合征(AIDS),通常是不可避免的进展,通常在症状发作后6个月内死亡。偶尔观察到患者存活超过1年,通常临床特征缓解。在这项研究中,我们通过比较7例症状发作后生存期超过12个月的患者和45例生存期较短的患者,确定了经活检证实的艾滋病相关PML患者生存期延长的预测因素。7例长期生存者中有5例(71.4%)以PML为AIDS表现,45例短期生存者中有8例(17.8%)以PML为AIDS表现。长期存活者的CD 4 T淋巴细胞计数明显较高,7例中有3例(42.9%)计数超过300个细胞/mm 3,而23例短期存活者中只有1例(4.3%)。在6例长期存活者中的3例(50%)和45例短期存活者中的4例(8.9%)中观察到放射学成像的对比度增强。在任何短期存活者中均未观察到神经功能恢复和影像学改善,但在5例(71.4%)长期存活者中观察到。治疗方式和生存率之间没有关联。艾滋病合并PML患者长期生存的预测因素包括作为艾滋病先兆表现的PML、疾病发作时CD 4 T淋巴细胞计数升高、计算机断层扫描或磁共振成像病变增强以及神经功能恢复的证据。
Progressive multifocal leukoencephalopathy (PML) complicating the acquired immunodeficiency syndrome (AIDS) is typically inexorably progressive with death usually occurring within 6 months of symptom onset. Occasional patients have been observed to survive longer than 1 year, often with remission of clinical features. In this study, we identify predictive factors for prolonged survival in patients with biopsy proven, AIDS-associated PML, by comparing 7 patients with survival exceeding 12 months from symptom onset with 45 patients with shorter survivals. PML was the presenting manifestation of AIDS in 5 (71.4%) of 7 long-term survivors compared with 8 (17.8%) of 45 short-term survivors. CD4 T-lymphocyte counts were substantially higher in the long-term survivors, with 3 (42.9%) of 7 having counts exceeding 300 cells/mm(3) in comparison with only 1 (4.3%) of 23 short-term survivors. Contrast enhancement on radiographic imaging was observed in 3 (50%) of 6 long-term survivors in comparison with 4 (8.9%) of 45 short-term survivors. Neurological recovery and radiographic improvement were not observed in any short-term survivors but were seen in 5 (71.4%) long-term survivors. There was no association between treatment modalities and survival. Predictors of longterm survival in AIDS patients with PML include PML as the heralding manifestation of AIDS, high CD4 T-lymphocyte count at disease onset, lesion enhancement on computed tomographic scan or magnetic resonance imaging, and evidence of recovery of neurological function.