[citation needed], Waldenström macroglobulinemia is characterized by an uncontrolled clonal proliferation of terminally differentiated B lymphocytes.

This is known as hyperviscosity, and it makes it difficult for all of your organs and tissues to function properly. et al.

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For those WM patients presenting with disease not requiring immediate disease control, several options can be considered and should ideally take into account specific disease-controlling objectives. Hyperviscosity syndrome is less frequently seen today as more patients are diagnosed and treated earlier.

Update on recommendations for assessing response from the Third International Workshop on Waldenstrom's Macroglobulinemia. The morbidity associated with WM is typically mediated by tissue infiltration by neoplastic cells, the physicochemical and immunologic properties of the monoclonal IgM, or both. "Mayo," "Mayo Clinic," "MayoClinic.org," "Mayo Clinic Healthy Living," and the triple-shield Mayo Clinic logo are trademarks of Mayo Foundation for Medical Education and Research.
B cells play a key role in the body's immune system. Waldenstrom macroglobulinemia is considered a type of non-Hodgkin's lymphoma. The IgM flare may last for several weeks, and even months, and does not per se herald treatment failure.54,62  The use of rituximab is best avoided as single-agent therapy in patients with high IgM levels because in 2 studies considerably lower response rates were observed in those patients with higher serum IgM levels (> 4000 mg/dL).64,65. Select one or more newsletters to continue. Last medically reviewed on January 7, 2016, Waldenstrom macroglobulinemia (WM) is a rare blood cancer.

Most of the patients had advanced WM.

This work was supported by the Peter and Helen Bing Fund for Waldenstrom Macroglobulinemia, the Linda and Edward Nelson Fund, the Bailey Family Fund for Waldenstrom Macroglobulinemia, and the International Waldenstrom Macroglobulinemia Foundation. Monoclonal gammopathy can be detected by the M-spike, or monoclonal spike, on serum protein electrophoresis, and serum immunofixation is used to identify the immunoglobulin M (IgM) heavy chain and the type of light chain involved.5 M-spikes restricted to the kappa-light chain were more frequently reported in patients diagnosed with WM.5 To help further differentiate WM from other lymphoplasmacytic lymphomas, a bone marrow biopsy, immunophenotyping by flow cytometry, and cytogenetic analysis are necessary. The abnormal white blood cells produce a protein that accumulates in the blood, impairs circulation and causes complications. This occurs when the free light chains produced by the lympho-plasmacellular clone, which underlies both WM and IgM-MGUS, turn out to be misfolded. Make a donation. The World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: Report of the Clinical Advisory Committee meeting, Airlie House, Virginia, November, 1997. One such cell is the B lymphocyte, which is also known as a B cell. A low level of neutrophils (a specific type of white blood cell) may also be found in some individuals with WM.

The addition of rituximab to front-line therapy with CHOP (R-CHOP) results in a higher response rate and longer time to treatment failure in patients with lymphoplasmacytic lymphoma: results of a randomized trial of the German Low-Grade Lymphoma Study Group (GLSG).
[15][16] A mutation in gene MYD88 has been found to occur frequently in patients.

Indeed, there can be great heterogeneity among patients between their respective serum IgM levels and bone marrow involvement (Figure 1). [51], One recent investigation showed that a population of cells, lacking both B-cell and plasma cell markers, has characteristics of cancer-initiating cells in Waldenström's macroglobulinemia. A warm bath collection should be considered for those patients suspected of having cryoglobulinemia to avoid underestimation of the serum IgM levels.

A Biologic activity of cold-reacting autoantibodies (first of two parts). As a third-line approach to the salvage therapy of WM, alemtuzumab can be also be considered.34,35  Alemtuzumab targets CD52, which is widely expressed on both bone marrow WM cells as well as on mast cells, which provide growth and survival signals to WM cells.77,78  As part of a WMCTG effort,79  28 patients with LPL (27 with WM) were treated with alemtuzumab. Search for other works by this author on: Clinicopathological definition of Waldenström's macroglobulinemia: Consensus Panel Recommendations from the Second International Workshop on Waldenström's macroglobulinemia. [53] Based on this study, the Food and Drug Administration approved ibrutinib for use in WM in 2015.

CT scans of the chest, abdomen, and pelvis should be obtained at time of diagnosis to properly stage the patient.32  Up to 20% of WM patients may have extramedullary disease, and CT scans offer an opportunity to assess for adenopathy, splenomegaly, and for other extramedullary disease sites. The cause of this disease is unknown. CD5, CD10, CD23 expression in Waldenstrom's macroglobulinemia. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2017. MJ Overall response rates with 4 weekly infusions of rituximab are 20% to 30%,64-66  whereas the use of extended-dose rituximab (ie, 4 weekly infusions followed by 4 more weekly infusions at week 12) has been associated with higher overall response rates (40%–50%).67,68  Polymorphisms in position 158 of the CD16 (FcγRIIIA-158) receptor have been shown in WM and related indolent lymphomas to predict response.69  In WM patients, a 4-fold higher rate of response has been reported among WM patients bearing the V/V or V/F polymorphism versus F/F.

Certain cells in the bone marrow of people with WM may release too much of a hormone involved in cell growth.

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