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

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.
AuthorsMeletios A Dimopoulos, Efstathios Kastritis, Roger G Owen, Robert A Kyle, Ola Landgren, Enrica Morra, Xavier Leleu, Ramón García-Sanz, Nikhil Munshi, Kenneth C Anderson, Evangelos Terpos, Irene M Ghobrial, Pierre Morel, David Maloney, Mathias Rummel, Véronique Leblond, Ranjana H Advani, Morie A Gertz, Charalampia Kyriakou, Sheeba K Thomas, Bart Barlogie, Stephanie A Gregory, Eva Kimby, Giampaolo Merlini, Steven P Treon
JournalBlood (Blood) Vol. 124 Issue 9 Pg. 1404-11 (Aug 28 2014) ISSN: 1528-0020 [Electronic] United States
PMID25027391 (Publication Type: Journal Article, Review)
Chemical References
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived
  • Antineoplastic Agents
  • Boronic Acids
  • Immunologic Factors
  • Nitrogen Mustard Compounds
  • Pyrazines
  • Rituximab
  • Bortezomib
  • Bendamustine Hydrochloride
  • Everolimus
  • Vidarabine
  • ofatumumab
  • fludarabine
  • Sirolimus
Topics
  • Antibodies, Monoclonal (therapeutic use)
  • Antibodies, Monoclonal, Humanized
  • Antibodies, Monoclonal, Murine-Derived (therapeutic use)
  • Antineoplastic Agents (therapeutic use)
  • Bendamustine Hydrochloride
  • Boronic Acids (therapeutic use)
  • Bortezomib
  • Clinical Trials as Topic
  • Consensus Development Conferences as Topic
  • Disease Progression
  • Everolimus
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunologic Factors (therapeutic use)
  • Nitrogen Mustard Compounds (therapeutic use)
  • Pyrazines (therapeutic use)
  • Rituximab
  • Salvage Therapy
  • Sirolimus (analogs & derivatives, therapeutic use)
  • Treatment Outcome
  • Vidarabine (analogs & derivatives, therapeutic use)
  • Waldenstrom Macroglobulinemia (therapy)

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