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- W2408643267 abstract "Objective: To report a rare case of Bing-Neel syndrome during ibrutinib monotherapy. Background: Bing-Neel syndrome (BNS) -- direct central nervous syetm (CNS) infiltration by malignant lymphoid cells -- is a rare complication of Waldenstrom’s macroglobulinemia (WM). A standard therapy has not been established as only case reports describing therapeutic strategies are available. No data have been published on the efficacy of ibrutinib, a small molecule inhibitor of Bruton’s tyrosine kinase, in BNS. Methods: Case report. Results: Over the course of six months, a 52-year-old gentleman with WM developed a neurologic syndrome comprised of headache, shuffling gait, precipitate micturition, visual disturbances (“blue haze around lights”), and memory difficulties. He was found to have grade IV papilledema. Neuroimaging studies showed communicating hydrocephalus. Flow cytometry of cerebrospinal fluid (CSF) identified IgM kappa-restricted monoclonal lymphoplasmacytoid cells. He received whole brain radiation followed by ibrutinib plus alternating intrathecal methotrexate, cytarabine, and rituximab. At five months, he achieved a complete response based on CSF analysis and MRI. He was lost to follow up after two intrathecal maintenance cycles but continued daily ibrutinib. Three months later, he presented with frequent falls, altered sensorium, and a possible seizure. MRI brain revealed numerous periventricular enhancing mass lesions with slowed water diffusion consistent with BNS. There was no evidence of systemic or meningeal relapse. Conclusions: This case of BNS in a patient with WM successfully treated with ibrutinib raises the question whether the CNS may have served as a sanctuary site for the lymphoplasmacytic clone. Ibrutinib has a low CSF-to-plasma concentration ratio (1-7[percnt]) and its efficacy in the treatment or prevention of BNS remains undetermined. Combination with other therapies may be required. Disclosure: Dr. Chan has nothing to disclose. Dr. Baehring has nothing to disclose. Dr. Becker has nothing to disclose." @default.
- W2408643267 created "2016-06-24" @default.
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- W2408643267 date "2016-04-05" @default.
- W2408643267 modified "2023-09-23" @default.
- W2408643267 title "Bing-Neel Syndrome in Waldenstrom’s Macroglobulinemia Treated with Ibrutinib: The Central Nervous System as a Sanctuary Site? (P6.288)" @default.
- W2408643267 hasPublicationYear "2016" @default.
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