P14.26 Autoimmune disease-associated primary CNS lymphoma: Meta-analysis and review of literature

Abstract

BACKGROUND: Recent studies suggest a relatively high prevalence of autoimmune diseases (AD) among primary CNS lymphoma (PCNSL) patients, however the literature is limited to case reports. To gain a better understanding of AD-associated PCNSL we reviewed all previous cases described in the literature. MATERIAL AND METHODS: We mined the MEDLINE database using the search terms 'central nervous system lymphoma' or 'CNS lymphoma' along with AD-related terms, such as 'immunosuppression', 'autoimmune', the name of various AD, or commonly prescribed immunosuppressants. We selected 35 records for qualitative synthesis of data. We identified 43 AD-associated PCNSL in the literature and added five unpublished cases from our institution. Clinical, imaging and outcome data were collected. RESULT(S): Most prevalent ADs were systemic lupus erythematosus (22.9%), multiple sclerosis (18.8%) and myasthenia gravis (14.6%). Male-to-female ratio was 1:2 and median age at diagnosis was 57 years (range: 2.5-88). Most common immunosuppressants included prednisone (58.7%), mycophenolate (43.5%), and azathioprine (41.3%). Median interval from diagnosis of AD until diagnosis of PCNSL was 96 months (range: 11-360). Lesions typically localized to the hemispheres (64.1%) and displayed peripheral contrast enhancement (75.8%). Histology revealed diffuse large-B-cell lymphomas (76.7%) and Epstein-Barr virus (EBV) positivity (78.4%). Treatment included reduction of immunosuppression (100%) and chemotherapy (85.4%) in most cases. Median overall survival was 31 months. CONCLUSION(S): AD-associated PCNSL are characterized by peripheral contrast enhancement on imaging and EBV positivity. AD that require severe immunosuppression appear over-represented. Median interval of immunosuppression and overall survival seem longer than in other immunodeficiency-associated PCNSL.