Bing-Neel Syndrome: A Case Report and Systematic Review of Clinical Manifestations, Diagnosis, and Treatment Options

• Published in: Clinical lymphoma & myeloma Vol. 9; no. 6; pp. 462 - 466
• Main Authors: Grewal, Jaspreet S., Brar, Preetkanwal K., Sahijdak, Walter M., Tworek, Joseph A., Chottiner, Elaine G.
• Format: Journal Article
• Language: English
• Published: United States 01.12.2009
• Subjects: Aged; Brain - pathology; Central Nervous System Diseases - diagnosis; Central Nervous System Diseases - etiology; Central Nervous System Diseases - therapy; CNS; Cranial Irradiation; Humans; Lymphoplasmacytic lymphoma; Lymphoplasmacytoid; Male; Malignant lymphoid infiltration; Neurologic complications; Plasma Cells - pathology; Rituximab; Syndrome; Waldenstrom Macroglobulinemia - cerebrospinal fluid; Waldenstrom Macroglobulinemia - complications; Waldenström macroglobulinemia; Waldenström's macroglobulinemia; Malignant lymphoid infiltration; Waldenström's macroglobulinemia; Lymphoplasmacytic lymphoma; Rituximab; CNS; Lymphoplasmacytoid; Neurologic complications; Waldenström macroglobulinemia
• ISSN: 1557-9190; 1938-0712
• DOI: 10.3816/CLM.2009.n.091

Bing-Neel syndrome is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia. We report a case of a 69-year-old white man with a 10-year history of WM. He was diagnosed with Bing-Neel syndrome based on magnetic resonance imaging and pathology studies of CSF. In addition, a comprehensive review of the reported cases of Bing-Neel syndrome in the up-to-date English-language literature was performed. Our patient underwent successful treatment with cranial radiation and intrathecal chemotherapy. He has been in clinical and pathologic remission for 3 years following the completion of his treatment. Based on our literature review, we also summarize and discuss clinical manifestations, diagnosis, and treatment options for Bing-Neel syndrome. Bing-Neel syndrome is a rare and potentially treatable complication of WM. Patients with a history of WM presenting with neurologic symptoms should be evaluated for possible Bing-Neel syndrome. Cranial radiation therapy alone or in combination with intrathecal chemotherapy is more likely to achieve sustainable remission than intrathecal chemotherapy alone.