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. Abstract: 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.
