Autoimmune disease-related primary CNS lymphoma: systematic review and meta-analysis
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CLINICAL STUDY
Autoimmune disease‑related primary CNS lymphoma: systematic review and meta‑analysis Leon D. Kaulen1,2 · Philipp Karschnia1,3 · Jorg Dietrich4 · Joachim M. Baehring1,5 Received: 23 April 2020 / Accepted: 11 July 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Background Recent studies suggest a relatively high prevalence of autoimmune disorders (AD) among primary CNS lymphoma (PCNSL) patients, however, the literature is limited to case reports. To gain a better understanding of AD-PCNSL we reviewed and analyzed all cases described in the literature. Methods We searched the MEDLINE database using the search terms ‘central nervous system lymphoma’ or ‘CNS lymphoma’ along with AD-related terms. We selected 39 records for qualitative synthesis of data and identified 50 AD-PCNSL. Clinical, imaging and outcome data were collected. Overall survival (OS) was analyzed with the Kaplan–Meier method. Univariate and multivariate analyses were performed using log rank test and Cox proportional hazard model. Results Most common AD were systemic lupus erythematosus (24%), multiple sclerosis (16%), and myasthenia gravis (14%). All patients had received immunosuppressants for their AD. Median interval from AD until PCNSL diagnosis was 108 months (range: 11–420). Male-to-female ratio was 0.42 and AD-PCNSL was diagnosed at a median age of 57 years (range: 2–88). On imaging lesions typically localized to the hemispheres (65%) and displayed peripheral enhancement (74%). Pathological evaluation revealed diffuse large-B-cell lymphoma (DLBCL) subtype (80%) and Epstein-Barr virus positivity (75%) in most AD-PCNSL. Median OS was 31 months. Age > 60 years (p = 0.014) was identified as a significant prognostic factor. Conclusions AD requiring immunosuppression appear over-represented in the population of PCNSL patients. Aggressive polychemotherapy can accomplish long term OS in AD-PCNSL comparable to immunocompetent patients. Age > 60 may serve as a prognostic factor. Keywords Primary CNS lymphoma · Autoimmune diseases · Epstein-barr virus · Immunosuppression · Brain tumor
Introduction Electronic supplementary material The online version of this article (https://doi.org/10.1007/s11060-020-03583-9) contains supplementary material, which is available to authorized users. * Joachim M. Baehring [email protected] 1
Department of Neurology, Yale School of Medicine, 15 York Street, New Haven, CT 06510, USA
2
Department of Neurology, Heidelberg University, Heidelberg, Germany
3
Department of Neurosurgery, Ludwig Maximilian University (LMU), Munich, Germany
4
Division of Neuro‑Oncology, Department of Neurology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, USA
5
Department of Neurosurgery, Yale School of Medicine, New Haven, USA
Several autoimmune disorders (AD) were previously shown to be potential risk factors for non-Hodgkin Lymphoma (NHL). The hazard correlates with AD type, choice of immunosuppressants and inflammatory activ
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