1. 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
- Author
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Daobin Zhou, Meng Zhang, Lu Zhang, Yan Zhang, Mei-xi Liu, Wei Zhang, Fan Jin, and Xiao-hang Liu
- Subjects
Adult ,Male ,medicine.medical_specialty ,Lymphoma ,Hyperreflexia ,Gastroenterology ,03 medical and health sciences ,0302 clinical medicine ,Maintenance therapy ,immune system diseases ,Pons ,hemic and lymphatic diseases ,Physiology (medical) ,Internal medicine ,Humans ,Medicine ,Inflammation ,Brain Diseases ,Receiver operating characteristic ,Chronic lymphocytic inflammation ,business.industry ,General Medicine ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Neurology ,030220 oncology & carcinogenesis ,Logistic analysis ,Hodgkin lymphoma ,Female ,Steroids ,Surgery ,Neurology (clinical) ,medicine.symptom ,business ,030217 neurology & neurosurgery ,After treatment - Abstract
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.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.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).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.
- Published
- 2019