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Blood, 15 June 2007, Vol. 109, No. 12, pp. 5096-5103. Prepublished online as a Blood First Edition Paper on February 15, 2007; DOI 10.1182/blood-2006-11-055012. This Article Full Text (PDF) All Versions of this Article: blood-2006-11-055012v1 109/12/5096    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Vijay, A. Articles by Gertz, M. A Search for Related Content PubMed PubMed Citation Articles by Vijay, A. Articles by Gertz, M. A Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted November 1, 2006 Accepted February 7, 2007 Waldenstrom Macroglobulinemia Arun Vijay and Morie A Gertz* Austin Medical Center, Mayo Health System, Austin, MN, United States Division of Hematology (M.A.G.), Mayo Clinic, Rochester, MN, United States * Corresponding author; email: gertz.morie{at}mayo.edu. In the past 36 months, new developments have occurred both in the understanding of the biology of Waldenstrom macroglobulinemia (WM) and in therapeutic options for WM. Here, we review the classification, clinical features, and diagnostic criteria of the disease. WM is a B-cell neoplasm characterized by lymphoplasmacytic infiltration of the bone marrow and a monoclonal immunoglobulin M (IgM) protein. The symptoms of WM are attributable to the extent of tumor infiltration and to elevated IgM levels. The most common symptom is fatigue attributable to anemia. The prognostic factors predictive of survival include the patient's age, 2-microglobulin, monoclonal protein, hemoglobin, and platelet count. Therapy is postponed for asymptomatic patients, and progressive anemia is the most common indication for initiation of treatment. The main therapeutic options include alkylating agents, nucleoside analogues, and rituximab. Studies involving combination chemotherapy are ongoing, and preliminary results are encouraging. No specific agent or regimen has been shown to be superior to another for treatment of WM. Novel agents such as bortezomib, perifosine, atacicept, oblimersen sodium, and tositumomab show promise as rational targeted therapy for WM. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: D. Provan, C. R.J. Singer, T. Baglin, and I. Dokal Waldenström macroglobulinaemia (WM) Oxford Handbook of Clinical Haematology, March 18, 2010; 3(1): med-9780199227396-div1-13 - med-9780199227396-div1-13. [Full Text] I. M. Ghobrial, M. Gertz, B. LaPlant, J. Camoriano, S. Hayman, M. Lacy, S. Chuma, B. Harris, R. Leduc, M. Rourke, et al. Phase II Trial of the Oral Mammalian Target of Rapamycin Inhibitor Everolimus in Relapsed or Refractory Waldenstrom Macroglobulinemia J. Clin. Oncol., March 10, 2010; 28(8): 1408 - 1414. [Abstract] [Full Text] [PDF] I. M. Ghobrial, F. Hong, S. Padmanabhan, A. Badros, M. Rourke, R. Leduc, S. Chuma, J. Kunsman, D. Warren, B. Harris, et al. Phase II Trial of Weekly Bortezomib in Combination With Rituximab in Relapsed or Relapsed and Refractory Waldenstrom Macroglobulinemia J. Clin. Oncol., March 10, 2010; 28(8): 1422 - 1428. [Abstract] [Full Text] [PDF] L. Gressier, C. Hotz, J.-D. Lelievre, A. Carlotti, M. Buffet, P. Wolkenstein, M. Bagot, G. Melica, and N. Ortonne Cutaneous Macroglobulinosis: A Report of 2 Cases Arch Dermatol, February 1, 2010; 146(2): 165 - 169. [Abstract] [Full Text] [PDF] H. T. Ngo, A. K. Azab, M. Farag, X. Jia, M. M. Melhem, J. Runnels, A. M. Roccaro, F. Azab, A. Sacco, X. Leleu, et al. Src Tyrosine Kinase Regulates Adhesion and Chemotaxis in Waldenstrom Macroglobulinemia Clin. Cancer Res., October 1, 2009; 15(19): 6035 - 6041. [Abstract] [Full Text] [PDF] M. V. Dhodapkar, A. Hoering, M. A. Gertz, S. Rivkin, J. Szymonifka, J. Crowley, and B. Barlogie Long-term survival in Waldenstrom macroglobulinemia: 10-year follow-up of Southwest Oncology Group-directed intergroup trial S9003 Blood, January 22, 2009; 113(4): 793 - 796. [Abstract] [Full Text] [PDF] X. Leleu, L. Xu, X. Jia, A. Sacco, M. Farag, Z. R. Hunter, A.-S. Moreau, H. T. Ngo, E. Hatjiharissi, A. W. Ho, et al. Endoplasmic reticulum stress is a target for therapy in Waldenstrom macroglobulinemia Blood, January 15, 2009; 113(3): 626 - 634. [Abstract] [Full Text] [PDF] X. Leleu, J. Soumerai, A. Roccaro, E. Hatjiharissi, Z. R. Hunter, R. Manning, B. T. Ciccarelli, A. Sacco, L. Ioakimidis, S. Adamia, et al. Increased Incidence of Transformation and Myelodysplasia/Acute Leukemia in Patients With Waldenstrom Macroglobulinemia Treated With Nucleoside Analogs J. Clin. Oncol., January 10, 2009; 27(2): 250 - 255. [Abstract] [Full Text] [PDF] J. Koshiol, G. Gridley, E. A. Engels, M. L. McMaster, and O. Landgren Chronic Immune Stimulation and Subsequent Waldenstrom Macroglobulinemia Arch Intern Med, September 22, 2008; 168(17): 1903 - 1909. [Abstract] [Full Text] [PDF] S. Poulain, I. Dervite, X. Leleu, V. Coiteux, P. Duthilleul, and P. Morel The IL6(-174G/C) polymorphism is a prognostic factor for survival after treatment initiation in Waldenstrom macroglobulinemia patients aged 65 years or less Haematologica, July 1, 2008; 93(7): 1109 - 1111. [Full Text] [PDF] H. T. Ngo, X. Leleu, J. Lee, X. Jia, M. Melhem, J. Runnels, A.-S. Moreau, N. Burwick, A. K. Azab, A. Roccaro, et al. SDF-1/CXCR4 and VLA-4 interaction regulates homing in Waldenstrom macroglobulinemia Blood, July 1, 2008; 112(1): 150 - 158. [Abstract] [Full Text] [PDF] X. Leleu, J. Eeckhoute, X. Jia, A. M. Roccaro, A.-S. Moreau, M. Farag, A. Sacco, H. T. Ngo, J. Runnels, M. R. Melhem, et al. Targeting NF-{kappa}B in Waldenstrom macroglobulinemia Blood, May 15, 2008; 111(10): 5068 - 5077. [Abstract] [Full Text] [PDF]

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