Waldenstrom Macroglobulinemia Staging 

Updated: Jan 04, 2018
  • Author: Joseph M Tuscano, MD; Chief Editor: Emmanuel C Besa, MD  more...
  • Print
Sections

Diagnostic Studies

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

  • History and physical examination with complete neurologic and ophthalmologic examination

  • Quantitative serum immunoglobulin G, immunoglobulin A, and immunoglobulin M (IgM) (total levels of serum immunoglobulins should be assessed with nephelometry)

  • Serum immunofixation

  • Twenty-four–hour urine collection for protein electrophoresis

  • Serum free light-chain assay

  • Bone marrow biopsy with cytogenetics, fluorescence in situ hybridization (FISH) (for lymphoma and myeloma), and flow cytometry (should include CD19, CD20, CD38, and CD138)

  • CT scanning of the chest, abdomen, and pelvis (to assess for lymphadenopathy and splenomegaly)

  • Complete blood cell (CBC) count and serum chemistries

  • Serum viscosity

  • Autoantibodies, Coombs test, cold agglutinins, cryoglobulins, anti–myelin-associated glycoprotein (MAG) antibodies (based on history and physical finding)

Staging/Prognostic Scoring System for Waldenstrom Macroglobulinemia/Lymphoplasmacytic Lymphoma

Each of the following positive prognostic factors is assigned a value of one, with the final sum used to assess prognostic score and predicted survival: [2]

  • IgM level >7 g/dL

  • Age >65 years

  • Hemoglobin level ≤11.5 g/dL

  • Platelet count ≤100,000/µL

  • Beta2-microglobulin level >3 µg/mL

The sum is then used to estimate prognosis and survival (see Table 1).

Table 1. Assessment of Prognostic Score and Predicted Survival (Open Table in a new window)

Score

Stratum

Median Survival (mo)

0-1a

Low

142

2b

Intermediate

96

>2

High

43

a Except age.

b Age >65 years is also intermediate risk.