Diagnosis and management of Waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines.
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.
Ansell
Stephen M
SM
Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. ansell.stephen@mayo.edu
Kyle
Robert A
RA
Reeder
Craig B
CB
Fonseca
Rafael
R
Mikhael
Joseph R
JR
Morice
William G
WG
Bergsagel
P Leif
PL
Buadi
Francis K
FK
Colgan
Joseph P
JP
Dingli
David
D
Dispenzieri
Angela
A
Greipp
Philip R
PR
Habermann
Thomas M
TM
Hayman
Suzanne R
SR
Inwards
David J
DJ
Johnston
Patrick B
PB
Kumar
Shaji K
SK
Lacy
Martha Q
MQ
Lust
John A
JA
Markovic
Svetomir N
SN
Micallef
Ivana N M
IN
Nowakowski
Grzegorz S
GS
Porrata
Luis F
LF
Roy
Vivek
V
Russell
Stephen J
SJ
Short
Kristen E Detweiler
KE
Stewart
A Keith
AK
Thompson
Carrie A
CA
Witzig
Thomas E
TE
Zeldenrust
Steven R
SR
Dalton
Robert J
RJ
Rajkumar
S Vincent
SV
Gertz
Morie A
MA
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
D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D007155:Immunologic Factors; D000069283:Rituximab