ArticleCureus2019
Development of Central Nervous System Vasculitis in a Patient with Waldenstrom Macroglobulinemia: A Rare Presentation with Poor Prognosis.
Article in Cureus, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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0 citing papers in PubMed, 1 citations in OpenAlex.
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Authors and funding
5 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Waldenstrom macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by the presence of monoclonal immunoglobulin M in serum. WM may present with neurologic complications involving the peripheral and central nervous systems (CNS) though CNS complications associated with WM are rare. We present a case of a 72-year-old male with an 18-month history of WM who experienced neurologic and constitutional symptoms indicative of WM progression over a three-week period while on rituximab maintenance therapy. The patient's symptoms were initially attributed to rituximab-induced asthenia though his clinical condition did not improve with rituximab discontinuation. Due to progressively worsening neurologic symptoms, the patient was re-evaluated and found to have multiple cerebral infarcts and increased serum cryoglobulin levels indicative of cryoglobulinema. The patient was promptly initiated on a combination regimen of high dose steroids, intravenous immunoglobulin (IVIG), and plasmapheresis but had a poor response. Brain biopsy revealed necrotizing vasculitis with dense intra- and peri-vascular CD3 positive T-cell infiltrates with mural necrosis. This is a unique case of WM complicated by type 1 cryoglobulinemia associated with CNS vasculitis that was unresponsive to active rituximab therapy; this case illustrates a poor prognosis of patients with CNS involvement in WM.
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