title: 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.
authors.affiliation: Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;
authors.forename: Meletios A
authors.initials: MA
authors.lastname: Dimopoulos
authors.affiliation: Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;
authors.forename: Efstathios
authors.initials: E
authors.lastname: Kastritis
authors.affiliation: St. James's Institute of Oncology, Leeds, United Kingdom;
authors.forename: Roger G
authors.initials: RG
authors.lastname: Owen
authors.affiliation: Mayo Clinic, Rochester, MN;
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.affiliation: National Cancer Institute, Bethesda, MD;
authors.forename: Ola
authors.initials: O
authors.lastname: Landgren
authors.affiliation: Department of Hematology, Niguarda Ca' Granda Hospital, Milan, Italy;
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
authors.affiliation: Service des Maladies du Sang, Hopital Huriez, Centre Hospitalier Régional Universitaire, Lille, France;
authors.forename: Xavier
authors.initials: X
authors.lastname: Leleu
authors.affiliation: Servicio de Hematología, Hospital Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca, Salamanca, Spain;
authors.forename: Ramón
authors.initials: R
authors.lastname: García-Sanz
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;
authors.forename: Nikhil
authors.initials: N
authors.lastname: Munshi
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;
authors.forename: Kenneth C
authors.initials: KC
authors.lastname: Anderson
authors.affiliation: Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;
authors.forename: Evangelos
authors.initials: E
authors.lastname: Terpos
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
authors.affiliation: Service d'Hématologie Clinique, Centre Hospitalier Schaffner, Lens, France;
authors.forename: Pierre
authors.initials: P
authors.lastname: Morel
authors.affiliation: Fred Hutchinson Cancer Research Center, Seattle, WA;
authors.forename: David
authors.initials: D
authors.lastname: Maloney
authors.affiliation: Medizinischen Klinik IV, Hospital of the Justus-Liebig-University Giessen, Germany;
authors.forename: Mathias
authors.initials: M
authors.lastname: Rummel
authors.affiliation: Assistance Publique-Hôpitaux de Paris Hôpital Pitiésalpêtrière, Université Pierre et Marie Curie Paris 6 GRC-11, Groupe de Recherche Clinique Hémopathie Lymphoïde, Paris, France;
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
authors.affiliation: Stanford Cancer Institute, Stanford, CA;
authors.forename: Ranjana H
authors.initials: RH
authors.lastname: Advani
authors.affiliation: Mayo Clinic, Rochester, MN;
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.affiliation: Royal Free Hospital, London, United Kingdom;
authors.forename: Charalampia
authors.initials: C
authors.lastname: Kyriakou
authors.affiliation: University of Texas MD Anderson Cancer Center, TX;
authors.forename: Sheeba K
authors.initials: SK
authors.lastname: Thomas
authors.affiliation: Myeloma Institute for Research and Therapy, University of Arkansas for Medical Sciences, Little Rock, AR;
authors.forename: Bart
authors.initials: B
authors.lastname: Barlogie
authors.affiliation: Rush University Medical Center, Chicago, IL;
authors.forename: Stephanie A
authors.initials: SA
authors.lastname: Gregory
authors.affiliation: Department of Hematology, Karolinska University Hospital, Solna, Sweden; and.
authors.forename: Eva
authors.initials: E
authors.lastname: Kimby
authors.affiliation: 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.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.affiliation: Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA;
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000069461:Bendamustine Hydrochloride; D001897:Boronic Acids / Q000627:therapeutic use; D000069286:Bortezomib; D002986:Clinical Trials as Topic; D003245:Consensus Statements as Topic; D018450:Disease Progression; D000068338:Everolimus; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D009588:Nitrogen Mustard Compounds / Q000627:therapeutic use; D011719:Pyrazines / Q000627:therapeutic use; D000069283:Rituximab; D016879:Salvage Therapy; D020123:Sirolimus / Q000031:analogs & derivatives / Q000627:therapeutic use; D016896:Treatment Outcome; D014740:Vidarabine / Q000031:analogs & derivatives / Q000627:therapeutic use; D008258:Waldenstrom Macroglobulinemia / Q000628:therapy*
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D001897:Boronic Acids; D007155:Immunologic Factors; D009588:Nitrogen Mustard Compounds; D011719:Pyrazines; D000069283:Rituximab; D000069286:Bortezomib; D000069461:Bendamustine Hydrochloride; D000068338:Everolimus; D014740:Vidarabine; C527517:ofatumumab; C024352:fludarabine; D020123:Sirolimus