Conflict of interest: Dr Gertz has received honoraria from Celgene Corporation (Summit, New Jersey), Millennium: The Takeda Oncology Company (Cambridge, Massachusetts), and The Binding Site Group Ltd (Birmingham, United Kingdom).
A Continuing Medical Education Series
Waldenström Macroglobulinemia: 2012 update on diagnosis, risk stratification, and management
Article first published online: 17 APR 2012
Copyright © 2012 Wiley Periodicals, Inc.
American Journal of Hematology
Volume 87, Issue 5, pages 503–510, May 2012
How to Cite
Gertz, M. A. (2012), Waldenström Macroglobulinemia: 2012 update on diagnosis, risk stratification, and management. Am. J. Hematol., 87: 503–510. doi: 10.1002/ajh.23192
- Issue published online: 16 APR 2012
- Article first published online: 17 APR 2012
- Manuscript Accepted: 2 MAR 2012
- Manuscript Received: 1 MAR 2012
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, β2 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 analog (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. Am. J. Hematol. 503–510, 2012. © 2012 Wiley Periodicals, Inc.