What are the diagnostic guidelines for Waldenstrom macroglobulinemia (WM)?

Updated: Feb 01, 2021
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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NCCN and ESMO guidelines concur that WM is diagnosed on the basis of histopathologic confirmation of bone marrow (BM) infiltration by monoclonal lymphoplasmacytic cells. [56, 55] BM infiltration is supported by immunophenotypic studies such as flow cytometry and/or immunohistochemistry. NCCN guidelines define the typical profile of WM cells as positive for slgM, CD19, CD20, CD22. [55] The typical profile defined by ESMO is positive for CD19, CD20, CD22 and CD79a on the lymphocytic component and CD38 on the plasmacytic component. [56]

NCCN guidelines also note that although WM lymphocytes are typically negative for CD5, CD10, and CD23, this should not exclude diagnosis, as 10-20% of cases express these proteins. [55]

Approximately 90% of patients with WM have the MYD88L265P mutation. Although this mutation alone is not diagnostic of WM, it may be useful for differentiating WM from other types of lymphoma and IgM multiple myeloma. [56, 55]

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