Objective:

To describe a rare case of Bing-Neel Syndrome (BNS) and elucidate the pertinent pathophysiology, neuroimaging, diagnostic, and treatment strategies.

Background:

Lymphoplasmacytic lymphoma, previously known as Waldenström macroglobulinemia, is malignant monoclonal gammopathy associated with IgM paraproteinemia. While the prototypical disease typically involves the reticuloendothelial system, BNS is a rare extramedullary variant with lymphoplasmacytic invasion of the central nervous system. BNS is usually a diagnosis of exclusion that confers a dismal prognosis. Fewer than 200 cases have been reported.

Design/Methods:

Case Report & Literature Review

Results:

75-year-old man with lymphoplasmacytic lymphoma adherent to rituximab monotherapy who presented 8 years into his disease course with subacute truncal ataxia, encephalopathy, and falls in the setting of failure-to-thrive and multi-organ carcinomatosis as evidenced by CT. ECOG Performance Status; 4. MRI Brain with contrast demonstrated multifocal lesions in the supratentorial white matter along with globular T2 FLAIR abnormalities in the cerebellum and brainstem. CSF differential via lumbar puncture revealed high protein (405), 45 nucleated cells (73% lymphocytes), and negative infectious findings. CSF cytopathology indicated atypical lymphoid pleocytosis and hypercellular fluid monolayers, favoring a neoplastic hematopoietic process. Confirmatory CSF flow cytometry showed undetectable surface light chain expression consistent with a CD5 negative, CD10 negative, B-cell lymphoproliferative disorder. His family declined further diagnostics (i.e. MYD88 genetic testing, gold-standard brain biopsy) and chemotherapy; they elected for comfort care after a corticosteroid regimen given his age and advanced disease burden.

Conclusions:

Further data on BNS are paramount given its rarity and morbidity. Prompt evaluation is crucial in offering options for patients and their families. This case report and literature review may guide further research on improving diagnostic paradigms, developing targeted therapies, and facilitating multidisciplinary care.