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Blood. Aug 28, 2014 Prepublished online Jul 15, 2015

Blood. Aug 28, 2014; 124(9): 1404–1411.
Prepublished online Jul 15, 2014


Treatment recommendations for patients with Waldenström macroglobulinemia (WM) and related disorders: IWWM-7 consensus.

Dimopoulos MA1, Kastritis E1, Owen RG2, Kyle RA3, Landgren O4, Morra E5, Leleu X6, García-Sanz R7, Munshi N8, Anderson KC8, Terpos E1, Ghobrial IM8, Morel P9, Maloney D10, Rummel M11, Leblond V12, Advani RH13, Gertz MA3, Kyriakou C14, Thomas SK15, Barlogie B16, Gregory SA17, Kimby E18, Merlini G19, Treon SP8.

Abstract
Waldenström 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, and neuropathy). Mature data show that rituximab combinations with cyclophosphamide/dexamethasone, bendamustine, or bortezomib/dexamethasone provided durable responses and are indicated for most patients. New monoclonal antibodies (ofatumumab), second-generation proteasome inhibitors (carfilzomib), mammalian target of rapamycin inhibitors, and Bruton's tyrosine kinase 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, reuse of a prior effective regimen may be appropriate. Autologous stem cell transplantation 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.

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