Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome

Bing Neel syndrome is a rare disease manifestation of Waldenstrom macroglobulinemia that results from infiltration of the central nervous system by malignant lymphoplasmacytic cells. In this guideline, we describe the clinical symptoms, as well as the appropriate laboratory and radiological studies that can aid in the diagnosis. The presentation of Bing Neel syndrome may be very diverse, and include headaches, cognitive deficits, paresis, and psychiatric symptoms. The syndrome can present in patients with known Waldenstrom macroglobulinemia, even in the absence of systemic progression, but also in previously undiagnosed patients. Diagnostic workup should include cerebral spinal fluid analysis with multiparameter flow cytometry to establish B-cell clonality, protein electrophoresis and immunofixation for the detection and classification of an monoclonal protein, as well as molecular diagnostic testing for immunoglobulin gene rearrangement and mutated MYD88. MRI of the brain and spinal cord is also essential. The second challenge is to expand our knowledge about the prognosis and treatment outcome. Prospective clinical trials in Bing Neel syndrome patients that employ uniform treatment along with appropriate laboratory cerebral spinal fluid assessments and standardized MRI protocols will be invaluable, and provide a significant step forward in delineating treatment outcome for this intriguing disease manifestation.