Current therapy guidelines for Waldenstrom's macroglobulinaemia.
Waldenstrom's macroglobulinaemia (WM) is a B-cell neoplasm in which bone marrow is infiltrated by lymphoplasmacytic cells that secrete monoclonal immunoglobulin M (IgM). More than a decade ago, specific criteria were agreed to define diagnosis and symptomatic disease requiring therapy; however, treatment recommendations change as new options emerge. Treatment decisions consider specific disease characteristics (burden of disease, IgM levels, presence of cytopenias) and patient characteristics (age, comorbidities, toxicity). Recently, the impact of specific mutations (in MYD88 and CXCR4) in response to specific therapies has been reported, and this may affect treatment decisions in the future. Chemo-immunotherapy combinations based on rituximab with cyclophosphamide/dexamethasone, bendamustine or bortezomib/dexamethasone are indicated for most patients. The BTK inhibitor ibrutinib was recently approved for patients with WM, and is a new option for selected newly diagnosed or relapsing patients. New B-cell receptor inhibitors, second-generation proteasome inhibitors and mammalian target of rapamycin inhibitors are promising; however, more data are needed from high-quality clinical trials.
Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.
Efstathios
E
Kastritis
Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece. Electronic address: mdimop@med.uoa.gr.
Meletios A
MA
Dimopoulos
D000225:Adenine / Q000031:analogs & derivatives; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D000069461:Bendamustine Hydrochloride / Q000627:therapeutic use; D000069286:Bortezomib / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D003520:Cyclophosphamide / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D009154:Mutation; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics / Q000276:immunology; D010880:Piperidines; D017410:Practice Guidelines as Topic; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D019718:Receptors, CXCR4 / Q000235:genetics / Q000276:immunology; D000069283:Rituximab / Q000627:therapeutic use; D008258:Waldenstrom Macroglobulinemia / Q000235:genetics / Q000276:immunology / Q000628:therapy*
C527276:CXCR4 protein, human; C507779:MYD88 protein, human; D053594:Myeloid Differentiation Factor 88; D010880:Piperidines; D011720:Pyrazoles; D011743:Pyrimidines; D019718:Receptors, CXCR4; C551803:ibrutinib; D000069283:Rituximab; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide; D000069461:Bendamustine Hydrochloride; D000225:Adenine
Bortezomib; Carfilzomib; Fludarabine; Ibrutinib; Rituximab