Diagnostic delay and outcome in immunocompetent patients with primary central nervous system lymphoma in Spain: a multicentric study

Diagnostic delay and outcome in immunocompetent patients with primary central nervous system lymphoma in Spain: a multicentric study
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DOI:
10.1007/s11060-020-03547-z
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发表时间:
2020-06-10
影响因子:
3.9
通讯作者:
Graus, F.
Graus, F.
中科院分区:
医学2区
文献类型:
--
作者:
Velasco, R.;Mercadal, S.;Graus, F.

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目的:评估西班牙原发性中枢神经系统淋巴瘤(PCNSL)免疫功能正常患者的管理。方法回顾性分析2005年至2014年西班牙27家医院经组织学证实的327例免疫功能正常的PCNSL患者。结果中位年龄64岁(范围:19-84岁;33% >= 70岁),男性占54%,59%诊断时表现状态(PS) >= 2。中位诊断延迟为47天(IQR 24-81)。诊断延迟bbb47天与PS >= 2 (OR 1.99; 95% CI 1.13-3.50; p = 0.016)和皮质类固醇治疗(OR 2.47; 95% CI 1.14-5.40; p = 0.023)相关,并且多年来没有改善。接受皮质类固醇治疗的患者(62%)有更高的额外活检风险(11.7%对4.0%,p = 0.04),但术前停用皮质类固醇并没有降低这种风险,反而增加了诊断延迟(64天对40天,p = 0.04)。整个系列的中位总生存期(OS)为8.9个月[95% CI 5.9-11.7],包括52例(16%)未接受治疗的患者,240例(73.4%)接受高剂量甲氨蝶呤(HD-MTX)化疗的患者的中位总生存期(OS)为14.1个月(95%CI 7.7-20.5)。bb0 = 70年的患者中位OS较短(4.1个月vs 13.4个月;p < 0.0001)。多变量分析确定年龄>= 65岁,PS >= 2,未治疗,诊断时的认知/精神症状是短期生存的独立预测因素。结论术前停用皮质类固醇不能降低活检阴性的风险,但会延迟诊断。在这项以社区为基础的研究中,只有73.4%的患者可以接受基于hd - mtx的化疗,OS仍然很差,特别是在老年患者>= 70岁。
Introduction To assess the management of immunocompetent patients with primary central nervous system lymphomas (PCNSL) in Spain. Methods Retrospective analysis of 327 immunocompetent patients with histologically confirmed PCNSL diagnosed between 2005 and 2014 in 27 Spanish hospitals. Results Median age was 64 years (range: 19-84; 33% >= 70 years), 54% were men, and 59% had a performance status (PS) >= 2 at diagnosis. Median delay to diagnosis was 47 days (IQR 24-81). Diagnostic delay > 47 days was associated with PS >= 2 (OR 1.99; 95% CI 1.13-3.50; p = 0.016) and treatment with corticosteroids (OR 2.47; 95% CI 1.14-5.40; p = 0.023), and it did not improve over the years. Patients treated with corticosteroids (62%) had a higher risk of additional biopsies (11.7% vs 4.0%, p = 0.04) but corticosteroids withdrawal before surgery did not reduce this risk and increased the diagnostic delay (64 vs 40 days, p = 0.04). Median overall survival (OS) was 8.9 months [95% CI 5.9-11.7] for the whole series, including 52 (16%) patients that were not treated, and 14.1 months (95%CI 7.7-20.5) for the 240 (73.4%) patients that received high-dose methotrexate (HD-MTX)-based chemotherapy. Median OS was shorter in patients >= 70 years (4.1 vs. 13.4 months; p < 0.0001). Multivariate analysis identified age >= 65 years, PS >= 2, no treatment, and cognitive/psychiatric symptoms at diagnosis as independent predictors of short survival. Conclusions Corticosteroids withdrawal before surgery does not decrease the risk of a negative biopsy but delays diagnosis. In this community-based study, only 73.4% of patients could receive HD-MTX-based chemotherapy and OS remains poor, particularly in elderly patients >= 70 years.