CNS Vasculitis Associated with Waldenström Macroglobulinemia

Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryog...

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Bibliographic Details
Main Authors: Tanawan Riangwiwat, Chris Y. Wu, Alberto S. Santos-Ocampo, Randal J. Liu, Aaron M. McMurtray, Beau K. Nakamoto
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2016/2510573
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Summary:Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient’s mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis. To our knowledge, CNS vasculitis in a WM patient, in the absence of cryoglobulins, has not been reported. Immunosuppression is the preferred treatment.
ISSN:2090-6668
2090-6676