Bing-Neel syndrome: A rare complication of waldenstrom macroglobulinemia

Central nervous system involvement is a rare complication of Waldenstrom macroglobulinemia (WM) accounting for 1-2% cases. This syndrome is called Bing-Neel syndrome (BNS) after the people who first described it in 1936. Although WM has a good prognosis, with the onset of this syndrome, most patients fare poorly with a high mortality rate. A 77/male, a known case of WM, presented with left upper limb weakness of 2 weeks duration. Magnetic resonance imaging brain showed diffuse pachymeningeal thickening along bilateral frontoparietal convexity. Biopsy showed lymphoplasmacytic lymphoma favoring a diagnosis of BNS. The patient was started on chemotherapy (rituximab + ibrutinib). At 3 months following diagnosis, the patient was admitted with Gram-negative septicemic shock and failed to recover from it. BNS is a rare complication of WM, associated with poor prognosis and an aggressive clinical course. It can occur during the course of treatment of WM, as was seen in this case. Accurate diagnosis with appropriate treatment plays a crucial role in patient management.