Efficacy and Safety of Zanubrutinib in IgA-secerning Lymphoplasmacytic Lymphoma

Author(s): Giulia Zamprogna, Anna Maria Frustaci, Marina Deodato, Heather Allewelt, Dirk Weber, Claudia Rigamonti, Alessandra Tedeschi

Non IgM-Lymphoplasmacytic Lymphoma (LPL) is a rare chronic lymph proliferative disorder with worse outcome with respect to classical LPL and Waldenstrom Macroglobulinemia. Therapeutic options are limited and differently from other indolent lymphomas no specific approved therapies are available. Here we present the case of a IgA-secreting LPL previously treated with 6 lines of therapies including immuno-chemotherapy, proteasome inhibitor and radiotherapy. At the last relapse this patient was successfully treated with zanubrutinib monotherapy within the BGB-311-AU-003 trial. Treatment with this BTK inhibitor showed to be well tolerated and led to a rapid and still ongoing response at 4 years follow up.