Waldenström macroglobulinemia: 2011 update on diagnosis, risk stratification, and management.

Abstract:

DISEASE OVERVIEW:Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy. DIAGNOSIS:Presence of IgM monoclonal protein associated with 10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. RISK STRATIFICATION:Age, hemoglobin level, platelet count, b2-microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis. RISK ADAPTED THERAPY:Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab-based therapy is used in virtually all US patients with WM and can be combined with alkylating agent or purine nucleoside analogue, or both. The preferred Mayo Clinic nonstudy therapeutic induction is rituximab, cyclophosphamide, and dexamethasone. Future stem cell transplantation should be considered in induction therapy selection. MANAGEMENT OF REFRACTORY DISEASE:Bortezomib, thalidomide, lenalidomide, and bendamustine have all been shown to have activity in WM. Given WM’s natural history, reduction of complications will be a priority for future treatment trials.

journal_name

Am J Hematol

authors

Gertz MA

doi

10.1002/ajh.22014

subject

Has Abstract

pub_date

2011-05-01 00:00:00

pages

411-6

issue

5

eissn

0361-8609

issn

1096-8652

journal_volume

86

pub_type

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