Background Vasculitis as a complication of lymphoproliferative diseases is relatively uncommon. Some cases of primary central nervous system vasculitis (PCNSV) have been described in association with lymphoma, particularly Hodgkin's lymphoma (HL).
Objectives The aim of this study is to report the presenting clinical, laboratory, and imaging features of patients with PCNSV associated with lymphoma and compare them with the characteristics of patients with PCNSV without lymphoma.
Methods We reviewed all patients diagnosed with vasculitis and lymphoma seen at the Mayo Clinic, Rochester, MN over the 32 year period of 1982–2014. 10 patients had PCNSV in association with lymphoma. We also used our updated cohort of 158 consecutive patients with PCNSV without lymphoma who were examined at Mayo Clinic over a 29-year period from 1983 to 2011. The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography, or both. Clinical data were collected.
Results 10/168 (5.9%) patients had PCNSV associated with lymphoma: 6 with HL and 4 with non-HL. PCNSV diagnosis was established by cerebral biopsy in 8 patients, and by cerebral arteriogram in 2. Two patients had spinal cord involvement. A granulomatous inflammatory histologic pattern was found in all 8 patients, accompanied by vascular deposits of β-amyloid peptide in 2. In 7 patients (5HL and 2NHL) neurologic symptoms preceded lymphoma diagnosis and subsequent medical workup revealed lymphoma.The 10 patients with lymphoma were compared with the 158 patients with PCNSV without lymphoma. The patients with lymphoma were more frequently males (80% vs 44%, p=0.04). No significant differences in the clinical manifestations at presentation and CSF findings were observed in the two groups, although cognitive dysfunction was more frequent in patients with lymphoma (80% vs 53%), while visual field deficit and intracranial hemorrhage less frequent (0% vs 19%, and 0% vs 10%, respectively). Systemic manifestations were infrequent in both groups (10% vs 9.5%). More patients with lymphoma showed meningeal gadolinium enhancing lesions at MRI (50% vs 19%, p=0.03). The frequencies of PCNSV relapse (20% vs 28.4%) and patients not requiring therapy at last follow-up (30% vs 25.5%) were similar in both groups. Patients with lymphoma had a higher frequency of poor outcome (modified Rankin disability score ≥4) at last followup (60% vs 21.5%, p=0.01). Considering all 168 patients, univariate Cox proportional hazards modeling showed an increased mortality rate in those with increasing age [hazard ratio (HR) 1.3], lymphoma (HR 3.9), cerebral infarction on MRI (HR 3.4), and angiographic large vessel involvement (HR 3.9), while mortality rate was lower in those with gadolinium–enhanced lesions on MRI (HR 0.3).
Conclusions PCNSV is associated with a diagnosis of lymphoma in 5.9% of PCNSV cases. Neurological manifestations of PCNSV may precede the diagnosis of lymphoma. Clinical characteristics of those with lymphoma were similar to those without lymphoma, however they had a more severe cerebral vasculitis with increased neurological disability and mortality.
Disclosure of Interest None declared