Zanubrutinib in Bing Neel syndrome: efficacy and tolerability
Anne‐Marie L. Becking, Johannes P. M. van de Mortel, Oliver Tomkins, T.W.H. Flinsenberg, Nicole Japzon, Marie José Kersten, Jahanzaib Khwaja, Saskia Kuipers, Henriëtte Levenga, Sean McKeague, Stephen Opat, Ross Salvaris, Sherif Seif, Sheeba K. Thomas, Alexander F. J. E. Vrancken, Shirley D’Sa, Monique C. Minnema, Josephine M. I. Vos
Abstract
Waldenström macroglobulinemia (WM) is an uncommon type of B-cell non-Hodgkin lymphoma, with bone marrow infiltration by lymphoplasmacytic lymphoma (LPL) cells accompanied by an immunoglobulin M paraprotein [ 1 , 2 ]. In ~1% of patients with WM, LPL cells invade the central nervous system (CNS) [ 3 ]. This condition, called Bing Neel syndrome (BNS), has a highly diverse clinical and radiological presentation, depending on its localization within the nervous system [ 4 , 5 , 6 ]. Due to its rarity, data on BNS treatment are limited to retrospective studies. Moreover, treatment is challenging as many standard WM therapies do not sufficiently cross the blood-brain barrier to reach effective CNS concentrations. Cytostatic agents, including high-dose methotrexate (MTX) and cytarabine or purine analogues, have traditionally been the only treatment options for BNS [ 4 , 5 , 7 ]. However, these agents are associated with high toxicity.