意識障害で発症したWaldenströmマクログロブリン血症（Bing-Neel症候群）の一例

抄録

A 76-year-old woman was transferred to our hospital because of consciousness disturbance. She showed severe anemia, thrombocytopenia with rouleaux formation, and monoclonal IgM-κ paraproteinemia, and a bone marrow biopsy specimen revealed infiltration of lymphoplasmacytic lymphoma cells (CD20+, IgM+). Brain MRI performed on day 1 exhibited spotty high intensity lesions in the cerebral white matter in diffusion-weighted images. Based on a diagnosis of hyperviscosity syndrome associated with Waldenström macroglobulinemia, the patient was treated with cyclophosphamide, methylprednisolone, and rituximab, and her consciousness level was slightly improved. However, brain MRI on day 32 revealed expansion of the high intensity lesions, many of which were enhanced following gadolinium injection. She was diagnosed with Bing-Neel syndrome, which indicates direct lymphoplasmacytoid involvement in the central nervous system. In spite of the application of a systemic chemotherapy, her general condition and consciousness level slowly worsened, and the patient died 3 months later. Bing-Neel syndrome should be considered as one of the causes of consciousness disturbance in neurological emergency cases.