title: Treatment of patients with Waldenström macroglobulinaemia: clinical practice guidelines from the Myeloma Foundation of Australia Medical and Scientific Advisory Group.
abstract: Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of immunoglobulin M (IgM) paraprotein and bone marrow infiltration by clonal small B lymphocytes, plasmacytoid lymphocytes and plasma cells. The symptoms of WM are protean, often follow an asymptomatic phase and may include complications related to the paraneoplastic effects of IgM paraprotein. The revised 2016 World Health Organization classification includes the MYD88 L265P mutation, which is seen in >90% of cases, within the diagnostic criteria for WM. While treatment of WM has often been considered together with other indolent B cell lymphomas, there are unique aspects of WM management that require specific care. These include the unreliability of IgM and paraprotein measurements in monitoring patients prior to and after treatment, the lack of correlation between disease burden and symptoms and rituximab-induced IgM flare. Moreover, while bendamustine and rituximab has recently been approved for reimbursed frontline use in WM in Australia, other regimens, including ibrutinib- and bortezomib-based treatments, are not funded, requiring tailoring of treatment to the regional regulatory environment. The Medical and Scientific Advisory Group of the Myeloma Foundation Australia has therefore developed clinical practice guidelines with specific recommendations for the work-up and therapy of WM to assist Australian clinicians in the management of this disease.
authors.affiliation: Department of Haematology, Canberra Hospital, Canberra, Australian Capital Territory, Australia.|Australian National University, Canberra, Australian Capital Territory, Australia.|Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.
authors.forename: Dipti
authors.initials: D
authors.lastname: Talaulikar
authors.affiliation: Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|Department of Haematology, St Vincent's Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Constantine S
authors.initials: CS
authors.lastname: Tam
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Douglas
authors.initials: D
authors.lastname: Joshua
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: Joy Phoebe
authors.initials: JP
authors.lastname: Ho
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.|Department of Clinical Haematology and BMT, Royal Melbourne Hospital, Melbourne, Victoria, Australia.
authors.forename: Jeff
authors.initials: J
authors.lastname: Szer
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, St Vincent's Hospital, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Hang
authors.initials: H
authors.lastname: Quach
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.|Department of Haematology, The Alfred Hospital, Melbourne, Victoria, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Spencer
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: Simon
authors.initials: S
authors.lastname: Harrison
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.|School of Medicine, University of Queensland, Brisbane, Queensland, Australia.
authors.forename: Peter
authors.initials: P
authors.lastname: Mollee
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.|Department of Clinical Haematology and BMT, Royal Melbourne Hospital, Melbourne, Victoria, Australia.|Walter and Eliza Hall Institute of Medical Research, Melbourne, Victoria, Australia.
authors.forename: Andrew W
authors.initials: AW
authors.lastname: Roberts
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
authors.forename: Noemi
authors.initials: N
authors.lastname: Horvath
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Adelaide Hospital, Adelaide, South Australia, Australia.
authors.forename: Cindy
authors.initials: C
authors.lastname: Lee
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Faculty of Medical Sciences, University of Adelaide, Adelaide, South Australia, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Zannettino
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
authors.forename: Ross
authors.initials: R
authors.lastname: Brown
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia.
authors.forename: Bradley
authors.initials: B
authors.lastname: Augustson
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Queen Elizabeth Hospital, Adelaide, South Australia, Australia.
authors.forename: Wilfrid
authors.initials: W
authors.lastname: Jaksic
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.|Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
authors.forename: John
authors.initials: J
authors.lastname: Gibson
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, The Alfred Hospital, Melbourne, Victoria, Australia.
authors.forename: Anna
authors.initials: A
authors.lastname: Kalff
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Hobart Hospital, Hobart, Tasmania, Australia.|Faculty of Health, University of Tasmania, Hobart, Tasmania, Australia.
authors.forename: Anna
authors.initials: A
authors.lastname: Johnston
authors.affiliation: Department of Haematology, Concord Hospital, Sydney, New South Wales, Australia.
authors.forename: Judith
authors.initials: J
authors.lastname: Trotman
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Royal Darwin Hospital, Darwin, Northern Territory, Australia.
authors.forename: Akash
authors.initials: A
authors.lastname: Kalro
authors.affiliation: Department of Haematology, Southern Health, Melbourne, Victoria, Australia.|Faculty of Medicine, Nursing and Health Sciences, Monash University, Melbourne, Victoria, Australia.
authors.forename: George
authors.initials: G
authors.lastname: Grigoriadis
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.|Department of Haematology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
authors.forename: Chris
authors.initials: C
authors.lastname: Ward
authors.affiliation: Medical and Scientific Advisory Group, Myeloma Australia, Melbourne, Victoria, Australia.|Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia.
authors.forename: H Miles
authors.initials: HM
authors.lastname: Prince
mesh_terms: D000225:Adenine / Q000031:analogs & derivatives; D026683:Advisory Committees; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001315:Australia; D000069461:Bendamustine Hydrochloride / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D000069286:Bortezomib / Q000627:therapeutic use; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood*; D009154:Mutation; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics; D010880:Piperidines; D010950:Plasma Cells / Q000473:pathology; D017410:Practice Guidelines as Topic; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D012955:Societies, Medical; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis / Q000188:drug therapy*
chemical_list: D000970:Antineoplastic Agents; D007075:Immunoglobulin M; C507779:MYD88 protein, human; D053594:Myeloid Differentiation Factor 88; D010880:Piperidines; D011720:Pyrazoles; D011743:Pyrimidines; C551803:ibrutinib; D000069283:Rituximab; D000069286:Bortezomib; D000069461:Bendamustine Hydrochloride; D000225:Adenine
keywords: Waldenström macroglobulinaemia; ibrutinib, bendamustine; lymphoplasmacytic lymphoma; monoclonal gammopathy of undetermined significance; rituximab