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Area of Science:

  • Neurology
  • Oncology
  • Immunology

Background:

  • Primary CNS vasculitis (PCNSV) is a rare condition affecting blood vessels in the brain and spinal cord.
  • Lymphoma is a type of cancer affecting the lymphatic system.
  • The association between PCNSV and lymphoma is not well-understood, but may involve paraneoplastic syndromes.

Purpose of the Study:

  • To document clinical findings, treatment responses, and disease progression in patients with PCNSV associated with lymphoma.
  • To compare these patients with those who have PCNSV but not lymphoma.

Main Methods:

  • Retrospective review of 936 patients with vasculitis and lymphoma over 32 years.
  • Identification of 10 patients with both PCNSV and lymphoma.
  • Comparison with 158 patients with PCNSV without lymphoma.

Main Results:

  • 5.9% of PCNSV patients had lymphoma (6 Hodgkin, 4 non-Hodgkin).
  • Patients with lymphoma were more often male, showed increased leptomeningeal enhancement, and had greater neurologic disability.
  • No significant difference in treatment response was observed between groups.

Conclusions:

  • Lymphoma can be diagnosed concurrently with PCNSV.
  • This co-occurrence suggests a potential immunologic paraneoplastic mechanism linking the two conditions.