Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas. (August 2019)

• Record Type: Journal Article
• Authors: Zhang, Lu; Liu, Xiao-hang; Jin, Fan; Liu, Mei-xi; Zhang, Meng; Zhang, Yan; Zhou, Dao-bin; Zhang, Wei
• Is Part Of: Journal of clinical neuroscience. Volume 66(2019)
• Page Start: 156
• Page End: 164
• Publication Date: 2019-08
• Subjects: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids -- Lymphoma -- Risk factors -- Clinical characteristics; Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2019.04.022
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: Characteristics of CLIPPERS with or without lymphoma were summarized and compared. Hyperreflexia, elevated CSF protein and early relapse were risk factors of lymphoma. Disease relapses during high-dose steroids therapy can also indicate lymphoma. A worse survival rate was demonstrated in CLIPPERS patients associated with lymphoma. Abstract: Background: We studied patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma and measured risk factors suggestive of an underlying lymphoma and follow-up outcomes. Methods: CLIPPERS patients associated with or without lymphoma were included into this study. Clinical presentations were documented, risk factors suggestive of an underlying lymphoma were tested, and prognostic differences in terms of death were compared. Results: Ten patients had a diagnosis of CLIPPERS associated with lymphoma, with 6 B-cell non-Hodgkin lymphoma, 2 T-cell non-Hodgkin lymphoma and 2 Hodgkin lymphoma. Using multivariate logistic analysis, the following 3 independent risk factors were found to be related to a final diagnosis of lymphoma: hyperreflexia (HR 16.56; 95% CI 1.03–265.29; p = 0.032), elevated protein in CSF (HR 11.59; 95% CI 1.24–108.39; p = 0.047), and recurrences between 2 months and 1 year after treatment (HR 29.27; 95% CI 2.09–409.58; p = 0.012). The model calibration was satisfactory (p = 0.392 with the Hosmer-Lemeshow test), and the discrimination power was good (area under the receiver operating characteristic curve 0.921; p < 0.001, 95% CI 0.826–1.000). Patients with CLIPPERS associated with lymphoma had higher mortality rate and lymphoma was a significant predictor of total mortality (HR 0.040; 95% CI 0.006–0.262; p = 0.001). Conclusions: Hyperreflexia, elevated protein in CSF and recurrences between 2 months and 1 year after treatment are risk factors suggesting an underlying lymphoma. Relapses during high-dose steroids maintenance therapy can be indicative of lymphoma, too. Patients having CLIPPERS associated with lymphoma have a worse prognosis than those without lymphoma.