Abstract
Bing–Neel syndrome (BNS) is a rare complication of Waldenstrom macroglobulinemia (WM) defined by a lymphoplasmacytic infiltration of the central nervous system (CNS). Patients present with a range of neurologic symptoms of variable severity. Diagnosis requires a low threshold of suspicion and is considered in WM patients with unexplained neurologic symptoms. It can occur in the presence of quiescent serum markers of WM. Direct CNS tissue biopsy should be pursued if feasible and remains the gold standard for diagnosis. No standard of care treatment exists, but expert guidelines suggest intravenous chemotherapy in standard dose or high-dose regimens or use of oral ibrutinib. Consideration is also made for intravenous rituximab, intrathecal therapies, and autologous stem cell transplantation. Patient factors and tolerability should drive decisions regarding treatment choice in this arena, given a lack of data for standard frontline therapy.
Highlights
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder characterized by a monoclonal lymphoplasmacytic infiltrate in the bone marrow with production of circulating monoclonal IgM antibody
An infrequent and poorly characterized neurologic disorder in WM patients, known as Bing–Neel syndrome, can be diagnosed when the dura, meninges, brain, or CSF are infiltrated by lymphoplasmacytic lymphoma (LPL) tumor cells
First described in 1936, involvement of the central nervous system (CNS) by WM is eponymously titled as the Bing–Neel syndrome (BNS) [5]
Summary
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder characterized by a monoclonal lymphoplasmacytic infiltrate in the bone marrow with production of circulating monoclonal IgM antibody. Over 90% of patients exhibit the MYD88L265P activating somatic mutation which leads to NF-κB-dependent survival of LPL cells [3]. Clinical manifestations of WM result from either marrow infiltration by the LPL tumor cells or peripheral effects of the circulating monoclonal IgM. Marrow infiltration by the tumor cells leads to myelophthisic anemia and thrombocytopenia. Circulating IgM exerts deleterious effects through several mechanisms: (i) increased serum viscosity, (ii) deposition in end organs, and (iii) auto-antibody activity. An infrequent and poorly characterized neurologic disorder in WM patients, known as Bing–Neel syndrome, can be diagnosed when the dura, meninges, brain, or CSF are infiltrated by LPL tumor cells
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