Waldenstrom Macroglobulinemia Staging 

Updated: Dec 19, 2019
  • Author: Joseph M Tuscano, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Diagnostic Studies

Recommended diagnostic studies to evaluate a suspected diagnosis of Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma are as follows [1] :

  • History and physical examination
  • Review of systems (B symptoms, hepatosplenomegaly, lymphadenopathy, neuropathy, Raynaud syndrome, rash, dyspnea, hyperviscosity symptoms including mucosal bleeding, blurred vision, headaches and dizziness; include fundoscopic examination to evaluate for retinal-vein engorgement, flame-shaped hemorrhages, papilledema if IgM is high and hyperviscosity is suspected)
  • Complete blood count
  • Complete metabolic panel
  • Serum immunoglobulin levels (IgA, IgG, IgM)
  • Serum and urine electrophoresis with immunofixation
  • Serum  beta2-microglobulin level
  • Viral serology (hepatitis B and C viruses and HIV)
  • Bone marrow aspiration and biopsy with immunohistochemistry (IHC; required for diagnosis), flow cytometry (optional; consider if IHC not available), and testing for  MYD88L265P gene mutation
  • Computed tomography scan of the chest, abdomen, and pelvis (to assess for lymphadenopathy and hepatosplenomegaly; if clinically indicated and in all patients being considered for therapy)

Optional studies, if clinically indicated, include the following:

  • Cryoglobulins
  • Cold agglutinin titer
  • Serum viscosity
  • Screening for acquired von Willebrand disease
  • 24-h urine protein quantification
  • Serum free light chain assay
  • N-terminal pro b-type natriuretic peptide, cardiac troponins
  • Electromyogram, anti–myelin-associated globulin antibody (anti-MAG), anti-ganglioside M1 (anti-GM1); to be ordered in consultation with a neurologist

Staging/Prognostic Scoring System for Waldenstrom Macroglobulinemia/Lymphoplasmacytic Lymphoma

Kastritis et al developed and validated an updated international prognostic score system for Waldenström macroglobulinemia. [2]  The system uses the following criteria:

  • Age: ≤65 years: 0 points, 66-75 years: 1 point; ≥75 years: 2 points
  • Beta2-microglobulin >4 mg/L: 1 point
  • Lactate dehydrogenase >250 IU/L: 1 point
  • Serum albumin < 3.5 g/dL: 1 point

Based on the total score, the system classifies patients into 5 risk groups (see table 1 below).

Table 1. Prognostic scoring system for Waldenstrom macroglobulinemia (Open Table in a new window)

Score

Stage

3-year WM-related mortality

5-year overall survival

10-year overall survival

0

Very low

0%

95%

84%

1

Low

10%

86%

59%

2

Intermediate

14%

78%

37%

3

High

38%

47%

19%

4-5

Very high

48%

36%

9%

 

 

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