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% |
Tables
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% |
