Open AccessEfficacy of Zanubrutinib in the Treatment of Bing–Neel Syndrome
Author(s) -
Wong Jonathan,
Cher Lawrence,
Griffiths James,
Cohen Aileen,
Huang Jane,
Wang Lai,
Gregory Gareth,
Opat Stephen
Publication year - 2018
Publication title -
hemasphere
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 11
ISSN - 2572-9241
DOI - 10.1097/hs9.0000000000000155
Subject(s) - medicine , lymphoplasmacytic lymphoma , waldenstrom macroglobulinemia , rituximab , paraproteinemia , gammopathy , vincristine , cyclophosphamide , macroglobulinemia , gastroenterology , surgery , pathology , chemotherapy , lymphoma , immunology , multiple myeloma , monoclonal , antibody , monoclonal antibody
Bing–Neel syndrome (BNS) is a rare complication of Waldenresponse (PR) according to modified 6th IWWM response ström’s macroglobulinemia (WM) characterized by clonal lymphoplasmacytic cell infiltration of the CNS, sometimes in association with CSF hyperglobulinemia. Treatment approaches have been based on limited evidence such as case reports and series. Herein, we report on a patient with BNS who responded to zanubrutinib, a second-generation Bruton tyrosine kinase (BTK) inhibitor. A 75-year-old female was first diagnosed with asymptomatic WM in 2004 following an incidental finding of IgM lambda paraproteinemia measuring 11g/L. She did not require treatment until she presented in May 2014 with warm autoimmune hemolytic anemia requiring immunosuppression and red cell transfusion. At that time, her serum IgM paraprotein had reached 23g/L. Bone marrow biopsy demonstrated effacement of normal hemopoiesis by lymphoplasmacytic lymphoma (comprising 40% of bone marrow cellularity). She denied any hyperviscosity, neurological, or constitutional symptoms. The patient was initially treated with 6 cycles of rituximab, cyclophosphamide, vincristine, and prednisone in 2014. She achieved a substantial reduction in the paraprotein to 4g/L and normalization of the hemoglobin, consistent with a partial