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Blood. 2014 Aug 28;124(9):1404-11. doi: 10.1182/blood-2014-03-565135. Epub 2014 Jul 15.

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

Author information

1
Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;
2
St. James's Institute of Oncology, Leeds, United Kingdom;
3
Mayo Clinic, Rochester, MN;
4
National Cancer Institute, Bethesda, MD;
5
Department of Hematology, Niguarda Ca' Granda Hospital, Milan, Italy;
6
Service des Maladies du Sang, Hopital Huriez, Centre Hospitalier Régional Universitaire, Lille, France;
7
Servicio de Hematología, Hospital Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca, Salamanca, Spain;
8
Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;
9
Service d'Hématologie Clinique, Centre Hospitalier Schaffner, Lens, France;
10
Fred Hutchinson Cancer Research Center, Seattle, WA;
11
Medizinischen Klinik IV, Hospital of the Justus-Liebig-University Giessen, Germany;
12
Assistance Publique-Hôpitaux de Paris Hôpital Pitiésalpêtrière, Université Pierre et Marie Curie Paris 6 GRC-11, Groupe de Recherche Clinique Hémopathie Lymphoïde, Paris, France;
13
Stanford Cancer Institute, Stanford, CA;
14
Royal Free Hospital, London, United Kingdom;
15
University of Texas MD Anderson Cancer Center, TX;
16
Myeloma Institute for Research and Therapy, University of Arkansas for Medical Sciences, Little Rock, AR;
17
Rush University Medical Center, Chicago, IL;
18
Department of Hematology, Karolinska University Hospital, Solna, Sweden; and.
19
Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Department of Molecular Medicine, University of Pavia, Pavia, Italy.

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.

PMID:
25027391
PMCID:
PMC4148763
DOI:
10.1182/blood-2014-03-565135
[Indexed for MEDLINE]
Free PMC Article

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