What are the clinical guidelines for staging and risk assessment of Waldenstrom macroglobulinemia (WM)?

Updated: May 29, 2020
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Both guidelines concur that the initial workup for WM includes the following [55, 54] :

  • Complete blood cell (CBC) count
  • Serum chemistry
  • Beta-2 microglobulin (B2M)
  • Serum protein electrophoresis
  • IgM quantification
  • CT scans with contrast when possible

Although CXCR4 mutations are found in approximately 30% of patients with WM, testing for this genetic mutation is recommended only for patients considering treatment with ibrutinib. [55, 54]

A neurologist should be consulted for neuropathy, since it may not be associated with WM. Amyloidosis should be ruled out in patients presenting with nephrotic syndrome or unexplained cardiac issues. Amyloidosis is uncommon in patients with WM. If it is suspected, a fat aspirate stained with Congo red and cardiac and renal biomarkers should be evaluated. [55, 54]

A retinal examination is recommended in patients with hyperviscosity symptoms. [55, 54]

NCCN finds the following additional tests useful in selected patients [54] :

  • Serum viscosity
  • Hepatitis C testing and cryocrit for patients with suspected cryoglobulinemia; if cryocrit is positive, initial serum IgM test should be repeated and all subsequent IgM levels should be obtained in warm conditions
  • Hepatitis B testing, if treatment with rituximab is planned
  • Coagulation testing should be considered in patients with symptoms of excess bruising or bleeding; von Willebrand disease (VWD) testing is indicated only if clinical bleeding and bruising are present

The ESMO guidelines include the following additional recommendations:

  • Anemia should prompt consideration of Coombs testing, cold agglutinins, cryoglobulins, and iron status.
  • Risk assessment is currently based on the International Prognostic Scoring System for WM (IPSSWM).

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