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Myeloproliferative disorder of B-lymphocytes producing abnormal plasma cells that secrete excess IgM causing hyperviscosity (thickening) of blood. WM is defined as a lymphoplasmacytic lymphoma (9671/3) with bone marrow involvement and IgM of any concentration. A significant subset of lymphoplasmacytic lymphoma patients has WM.
Waldenstrom Macroglobulinemia (WM) is a subset of lymphoplasmacytic lymphoma.
Patients with WM have IgM (immunoglobulin M) in their blood and/or bone marrow. IgM is also called IgM monoclonal gammopathy. There will also be an increased number of lymphocytes in the blood. A familial disposition may exist in up to 20% of patients. It tends to be diagnosed at a younger age than other lymphoplasmacytic lymphomas.
Treatment of Waldenstrom macroglobulinemia may include the following: 1. Biologic (immuno) therapy with interferon 2. Chemotherapy 3. Plasmapheresis 4. Proteasome inhibitor 5. Watchful waiting
Definitive Diagnostic Methods
6q deletion in 50% of cases
Somatic hypermutations in V regions of IG genes (IgM), t(9;14)(p13;q32) in 50% of cases
Trisomy 4 in 20% of cases
WM has a homogenous gene expression profile other than 6q deletion, more similar to CLL and normal B-cells than to myeloma