IBCL-019: Waldenström Macroglobulinemia (WM) in a Public Health Service in Sao Paulo, Brazil

Abstract

Context: WM is a rare indolent non-Hodgkin lymphoma that mainly affects people over 60 years old. It is characterized by lymphoplasmacytic cells bone marrow infiltration and IgM gammopathy. Patients have asthenia, weight loss, mucosal bleeding, lymph node enlargement, splenomegaly and neurological changes resulting from hyperviscosity and/or cytopenias. Treatment goal is to relieve symptoms. Recently, targeted drugs have been used, which may increase its median overall survival in the next years. Notwithstanding these drugs are not equally available around the world. Objective: To recognize clinical, epidemiological profile and treatment evaluation of WM patients at Santa Casa de Sao Paulo, Brazil. Design: WM retrospective analysis cohort diagnosed between January 1, 2000 and December 31, 2016. Patients or other participants: We analyzed patients with WM diagnosis. HIV, HBV, and HCV positive serology patients were excluded. Results: There were 18 patients, 10 men/8 women, with 61 years old median age. Fifteen patients presented with splenomegaly; 14 had B symptoms at diagnosis. Median serum IgM was 3019 mg/dL; 8 patients were submitted to plasmapheresis because of symptomatic hyperviscosity syndrome. 70% were classified as High/Very-high risk and all patients were treated upfront. As 1st line treatment we used alkylant + prednisone in 12 patients; alkylant + prednisone + anthracycline in 3 patients; fludarabine in 2 patients and to 1 patient only prednisone. Less than 25% got CR/VGPR; 5.5% got PR and 72% were refractory. There were 2 deaths before 2nd line treatment for which we used fludarabine-based therapy in 9, alkylant + prednisone + anthracycline in 2 and alkylant + prednisone in 1 patient. Ten patients were dead before 3rd line therapy. Mean OS was 55 months. Hb under 9.5 g/dl and platelets under 120×103mm3 had statistically significant worse median OS. Conclusions: Our Waldenstrom macroglobulinemia cohort showed poor response to 1st line chemotherapy regimens. IPSS-WM showed that they arrive in severe clinical conditions making it a challenging treatment. The absence of new available target drugs might have resulted in bad 1st line response and a high relapse rate.