title: Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines.
abstract: Waldenström macroglobulinemia is a B-cell malignancy with lymphoplasmacytic infiltration in the bone marrow or lymphatic tissue and a monoclonal immunoglobulin M protein (IgM) in the serum. It is incurable with current therapy, and the decision to treat patients as well as the choice of treatment can be complex. Using a risk-adapted approach, we provide recommendations on timing and choice of therapy. Patients with smoldering or asymptomatic Waldenström macroglobulinemia and preserved hematologic function should be observed without therapy. Symptomatic patients with modest hematologic compromise, IgM-related neuropathy that requires therapy, or hemolytic anemia unresponsive to corticosteroids should receive standard doses of rituximab alone without maintenance therapy. Patients who have severe constitutional symptoms, profound hematologic compromise, symptomatic bulky disease, or hyperviscosity should be treated with the DRC (dexamethasone, rituximab, cyclophosphamide) regimen. Any patient with symptoms of hyperviscosity should first be treated with plasmapheresis. For patients who experience relapse after a response to initial therapy of more than 2 years' duration, the original therapy should be repeated. For patients who had an inadequate response to initial therapy or a response of less than 2 years' duration, an alternative agent or combination should be used. Autologous stem cell transplant should be considered in all eligible patients with relapsed disease.
authors: Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. ansell.stephen@mayo.edu
authors: Stephen M
authors: SM
authors: Ansell
authors: Robert A
authors: RA
authors: Kyle
authors: Craig B
authors: CB
authors: Reeder
authors: Rafael
authors: R
authors: Fonseca
authors: Joseph R
authors: JR
authors: Mikhael
authors: William G
authors: WG
authors: Morice
authors: P Leif
authors: PL
authors: Bergsagel
authors: Francis K
authors: FK
authors: Buadi
authors: Joseph P
authors: JP
authors: Colgan
authors: David
authors: D
authors: Dingli
authors: Angela
authors: A
authors: Dispenzieri
authors: Philip R
authors: PR
authors: Greipp
authors: Thomas M
authors: TM
authors: Habermann
authors: Suzanne R
authors: SR
authors: Hayman
authors: David J
authors: DJ
authors: Inwards
authors: Patrick B
authors: PB
authors: Johnston
authors: Shaji K
authors: SK
authors: Kumar
authors: Martha Q
authors: MQ
authors: Lacy
authors: John A
authors: JA
authors: Lust
authors: Svetomir N
authors: SN
authors: Markovic
authors: Ivana N M
authors: IN
authors: Micallef
authors: Grzegorz S
authors: GS
authors: Nowakowski
authors: Luis F
authors: LF
authors: Porrata
authors: Vivek
authors: V
authors: Roy
authors: Stephen J
authors: SJ
authors: Russell
authors: Kristen E Detweiler
authors: KE
authors: Short
authors: A Keith
authors: AK
authors: Stewart
authors: Carrie A
authors: CA
authors: Thompson
authors: Thomas E
authors: TE
authors: Witzig
authors: Steven R
authors: SR
authors: Zeldenrust
authors: Robert J
authors: RJ
authors: Dalton
authors: S Vincent
authors: SV
authors: Rajkumar
authors: Morie A
authors: MA
authors: Gertz
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001809:Blood Viscosity; D004359:Drug Therapy, Combination; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D010956:Plasmapheresis; D011379:Prognosis; D012008:Recurrence; D012307:Risk Factors; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007155:Immunologic Factors; D000069283:Rituximab