Diagnosing, imaging, and successfully treating a debilitating case of Bing–Neel syndrome: A multidisciplinary feat

Key Clinical Message We present a case of Bing–Neel syndrome a rare central nervous system lymphoplasmocytic lymphoma associated with Waldenstrom macroglobulinemia. Diagnosis should be considered in the context of unexplained neurological symptoms in the presence of macroglobulinemia. Waldenstroms m...

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Bibliographic Details
Published in:Clinical case reports 2023-05, Vol.11 (5), p.e7296-n/a
Main Authors: Kerley, Robert N., O'Donnell, Niamh, Lynott, Fiona, Mulcahy, Riona, Hennessy, Brian
Format: Article
Language:English
Subjects:
Bing–Neel syndrome
Biopsy
Case Report
Case reports
Chemotherapy
Conflicts of interest
Consent
diagnostic imaging
Flow cytometry
Funding
Immunotherapy
Kinases
Lymphoma
Medical prognosis
Neurological disorders
Patients
Remission (Medicine)
Waldenstrom macroglobulinemia
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Summary:Key Clinical Message We present a case of Bing–Neel syndrome a rare central nervous system lymphoplasmocytic lymphoma associated with Waldenstrom macroglobulinemia. Diagnosis should be considered in the context of unexplained neurological symptoms in the presence of macroglobulinemia. Waldenstroms macroglobulinaemia (WM) is a rare B‐cell lymphoma representing ~2% of all hematological malignancies. While most neurological complications of WM are secondary to the overproduction of immunoglobulin M (IgM), Bing‐Neel syndrome (BNS) is an extremely rare direct central nervous system (CNS) infiltration by malignant lymphoplasmocytic lymphoma (LPL) cells. Limited information on BNS exists in the literature with sparse case reports and case series. Here, we present a diagnostically challenging BNS case successfully treated with systemic chemoimmunotherapy and ibrutinib, with remarkable clinical response.
ISSN:2050-0904
2050-0904
DOI:10.1002/ccr3.7296