28(8):1408-14. The current treatment of waldenstrom's macroglobulinemia is primarily intended to treat symptoms and help prevent complications rather than treat the disease. Cancer. Bone-marrow plasma cell burden correlates with IgM paraprotein concentration in Waldenstrom macroglobulinaemia. Patients who do not show symptoms of waldenstrom's macroglobulinemia are often monitored without being treated. The NCCN Guidelines® are a consensus statement of its authors regarding their views of currently accepted approaches to treatment. [49]. [33] The principle behind management is that 80% of all IgM is confined to the intravascular space. [5] Patients can be observed carefully with periodic measurement of the M component, immunoglobulin, and serum viscosity. Treatment varied depending on local protocols. J Clin Oncol. Viscosity should be measured before and after plasmapheresis. Fludarabine plus cyclophosphamide and rituximab in Waldenström macroglobulinemia: an effective but myelosuppressive regimen to be offered to patients with advanced disease. Diseases & Conditions, 2002 Days 1, 4, 8, and 11: Bortezomib 1.3mg/m2 IV + dexamethasone 40mg IV. The genomic landscape of Waldenstrom macroglobulinemia is characterized by highly recurring MYD88 and WHIM-like CXCR4 mutations, and small somatic deletions associated with B-cell lymphomagenesis. Dhodapkar et al, in a study evaluating fludarabine response in previously untreated and previously treated patients, reported an overall response rate of 36%, with 3% of patients experiencing complete remission; the overall survival period was 84 months. Available at: http://www.nccn.org/professionals/physician_gls/pdf/waldenstroms.pdf. Patients who meet the diagnostic criteria for Waldenström macroglobulinemia (WM) on the basis of serum IgM monoclonal protein, bone marrow lymphoplasmacytic infiltration, or both but who do not have evidence of end-organ damage are considered to have indolent disease or smoldering Waldenström macroglobulinemia. Doris Ponce, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Hematology, American Society of Clinical OncologyDisclosure: Nothing to disclose. [Full Text]. 2019 Dec 5. Chemotherapy: This often is the most effective treatment, and a J Clin Pathol. [Full Text]. Days 1, 4, 8, and 11: Bortezomib 1.0 or 1.3mg/m2. [29] : Laboratory indications for initiation of therapy include the following Register now at no charge to access unlimited clinical news, full-length features, case studies, conference coverage, and more. a Pneumocystis jiroveci pneumonia (PJP) prophylaxis should be considered for patients receiving bendamustine/rituximab or fludarabine/cyclophosphamide/rituximab. 64(6):520-3. After entering remission, patients aged 70 years or younger who are potential candidates for autologous stem cell transplantation may be considered for stem cell harvest for future use. Blood. [Medline]. [Full Text]. AJR Am J Roentgenol. The addition of rituximab to front-line therapy with CHOP (R-CHOP) results in a higher response rate and longer time to treat failure in patients with lymphoplasmacytic lymphoma: results of a randomized trial of the German Low-Grade Lymphoma Study Group (GLSG). [Medline]. 5. We performed a prospective phase II study to clearly define the activity of rituximab in patients with this disease. 9(1):59-61. Allogeneic Transplantation for Relapsed Waldenström Macroglobulinemia and Lymphoplasmacytic Lymphoma. [Medline]. Most often, half of the volume or more should be removed to significantly lower the serum viscosity. Ghobrial IM, Gertz M, Laplant B, et al. Treatment recommendations for patients with Waldenström’s macroglobulinemia (WM) and related disorders: IWWM-7 consensus.

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