Diagnosis and management of waldenström macroglobulinemia: Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines

Stephen Maxted Ansell, Robert A. Kyle, Craig B. Reeder, Rafael Fonseca, Joseph R Mikhael, William G. Morice, Peter Leif Bergsagel, Francis K. Buadi, Joseph P. Colgan, David M Dingli, Angela Dispenzieri, Philip R. Greipp, Thomas Matthew Habermann, Suzanne R. Hayman, David J. Inwards, Patrick Bruce Johnston, Shaji K Kumar, Martha Lacy, John A. Lust, Svetomir Nenad MarkovicIvana Micallef, Grzegorz S Nowakowski, Luis F. Porrata, Vivek Roy, Stephen J Russell, Kristen E. Detweiler Short, Alexander Keith Stewart, Carrie A Thompson, Thomas Elmer Witzig, Steven R. Zeldenrust, Robert J. Dalton, S Vincent Rajkumar, Morie Gertz

Research output: Contribution to journalArticle

104 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)824-833
Number of pages10
JournalMayo Clinic Proceedings
Volume85
Issue number9
DOIs
StatePublished - 2010

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Waldenstrom Macroglobulinemia
Guidelines
Therapeutics
Immunoglobulin M
Plasmapheresis
Hemolytic Anemia
Lymphoid Tissue
Cyclophosphamide
Dexamethasone
Blood Proteins
Adrenal Cortex Hormones
B-Lymphocytes
Stem Cells
Bone Marrow
Transplants
Bone and Bones
Recurrence

ASJC Scopus subject areas

  • Medicine(all)

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Diagnosis and management of waldenström macroglobulinemia : Mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines. / Ansell, Stephen Maxted; Kyle, Robert A.; Reeder, Craig B.; Fonseca, Rafael; Mikhael, Joseph R; Morice, William G.; Bergsagel, Peter Leif; Buadi, Francis K.; Colgan, Joseph P.; Dingli, David M; Dispenzieri, Angela; Greipp, Philip R.; Habermann, Thomas Matthew; Hayman, Suzanne R.; Inwards, David J.; Johnston, Patrick Bruce; Kumar, Shaji K; Lacy, Martha; Lust, John A.; Markovic, Svetomir Nenad; Micallef, Ivana; Nowakowski, Grzegorz S; Porrata, Luis F.; Roy, Vivek; Russell, Stephen J; Detweiler Short, Kristen E.; Stewart, Alexander Keith; Thompson, Carrie A; Witzig, Thomas Elmer; Zeldenrust, Steven R.; Dalton, Robert J.; Rajkumar, S Vincent; Gertz, Morie.

In: Mayo Clinic Proceedings, Vol. 85, No. 9, 2010, p. 824-833.

Research output: Contribution to journalArticle

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abstract = "Waldenstr{\"o}m 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 Waldenstr{\"o}m 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.",
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AU - Reeder, Craig B.

AU - Fonseca, Rafael

AU - Mikhael, Joseph R

AU - Morice, William G.

AU - Bergsagel, Peter Leif

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AU - Dispenzieri, Angela

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AU - Habermann, Thomas Matthew

AU - Hayman, Suzanne R.

AU - Inwards, David J.

AU - Johnston, Patrick Bruce

AU - Kumar, Shaji K

AU - Lacy, Martha

AU - Lust, John A.

AU - Markovic, Svetomir Nenad

AU - Micallef, Ivana

AU - Nowakowski, Grzegorz S

AU - Porrata, Luis F.

AU - Roy, Vivek

AU - Russell, Stephen J

AU - Detweiler Short, Kristen E.

AU - Stewart, Alexander Keith

AU - Thompson, Carrie A

AU - Witzig, Thomas Elmer

AU - Zeldenrust, Steven R.

AU - Dalton, Robert J.

AU - Rajkumar, S Vincent

AU - Gertz, Morie

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