Treatment recommendations for patients with Waldenström's Macroglobulinemia (WM) and related disorders: consensus from the Seventh International Workshop on WM

Waldenstrom's macroglobulinemia (WM) is a distinct B-cell lymphoproliferative disorder for which clearly defined criteria for the diagnosis, initiation of therapy and treatment strategy have been proposed as part of the consensus panels of International Workshops on WM (IWWM). As part of the IWWM-7 and based on recently published and ongoing clinical trials the panels updated treatment recommendations. Therapeutic strategy in WM should be based on individual patient and disease characteristics (age, comorbidities, need for rapid disease control, candidacy for autologous transplantation, cytopenias, IgM-related complications (hyperviscosity, neuropathy). Mature studies show that rituximab combinations with cyclophosphamide/dexamethasone (DRC), or bendamustine (BR) or bortezomib/dexamethasone (BDR) provided durable responses and are indicated for most patients. New monoclonal antibodies (ofatumumab), second generation proteasome inhibitors (carfilzomib), mTOR inhibitors and BTK inhibitors, are promising and may expand future treatment options. A different regimen is typically recommended for relapsed or refractory disease. In selected patients with relapsed disease after long lasting remission re-use of a prior effective regimen may be appropriate. AutoSCT may be considered in young patients with chemosensitive disease and in newly diagnosed patients with very high-risk features. Active enrollment of patients with WM in clinical trials is encouraged.