Waldenstrom Macroglobulinemia Staging 

Updated: Nov 18, 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, organomegaly, hyperviscosity symptoms, neuropathy, Raynaud syndrome, rash, peripheral edema, skin abnormalities, dyspnea; include fundoscopic examination 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 (o assess fotlymphadenopathy and splenomegaly; 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 have 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; ≥76 years, 2 points
  • Beta2-microglobulin > 4 mg/L: 1 point
  • Lactate dehydrogenase ≥ 250 IU/L (upper limit of normal <  225 IU/L): 1 point
  • Serum albumin < 3.5 g/dL: 1 point

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

Table.  Prognostic score system for Waldenström macroglobulinemia (Open Table in a new window)

Point total

Risk group

3-year WM-related death rate (%)

10-year overall survival rate (%)

0

Very low

0

84

1 Low

10

59

2 Intermediate

14

37

3 High

38

19

4-5

Very high

48

9

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