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Case Rep Neurol Med. 2016;2016:2510573. Epub 2016 Oct 12.

CNS Vasculitis Associated with Waldenström Macroglobulinemia.

Author information

1
Department of Medicine, University of Hawaii, Honolulu, HI, USA.
2
Department of Rheumatology, Straub Clinics and Hospital, Honolulu, HI, USA.
3
Department of Oncology, Straub Clinics and Hospital, Honolulu, HI, USA.
4
Department of Neurology, Harbor-UCLA Medical Center, Torrance, CA, USA.
5
Department of Medicine, University of Hawaii, Honolulu, HI, USA; Department of Neurology, Straub Clinics and Hospital, Honolulu, HI, USA.

Abstract

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.

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