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CNS Vasculitis Associated with Waldenström Macroglobulinemia
Tanawan Riangwiwat1, Chris Y Wu1, Alberto S Santos-Ocampo2
1Department of Medicine, University of Hawaii, Honolulu, HI, USA.
Waldenström macroglobulinemia (WM) can cause central nervous system (CNS) vasculitis, leading to strokes. Immunosuppression effectively treated this rare WM complication without cryoglobulins.
Area of Science:
- Neurology
- Hematology
- Immunology
Background:
- Waldenström macroglobulinemia (WM) is a rare, indolent B-cell lymphoma characterized by IgM monoclonal gammopathy.
- Central nervous system (CNS) involvement in WM is uncommon and often associated with hyperviscosity or cryoglobulinemia.
Purpose of the Study:
- To report a unique case of CNS vasculitis in a patient with WM.
- To highlight the successful management of this rare complication.
Main Methods:
- Case presentation of a patient with WM presenting with ischemic strokes.
- Diagnostic workup excluding cryoglobulins and hyperviscosity syndrome.
- Treatment with immunosuppressive therapy (intravenous steroids and cyclophosphamide).
Main Results:
- The patient presented with multifocal acute cortical ischemic strokes.
- Cerebrospinal fluid and imaging confirmed CNS vasculitis.
- Immunosuppressive treatment led to stabilization of neurological deficits.
- Long-term follow-up (7 years) showed no recurrence and functional recovery.
Conclusions:
- CNS vasculitis can occur in WM even without cryoglobulins or hyperviscosity.
- Immunosuppression is an effective treatment strategy for CNS vasculitis in WM.
- This case expands the known spectrum of CNS complications in Waldenström macroglobulinemia.
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