What is the prognosis of Waldenström macroglobulinemia?

Updated: Feb 01, 2021
  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Waldenström macroglobulinemia is an indolent disorder, and patients survive for a median of approximately 78 months. Kaplan-Meier survival curves of patients with Waldenström macroglobulinemia do not show a plateau. [15]

Different studies have been performed to assess prognosis. Patients with a nodular type of bone marrow involvement tend to do better than those with diffuse involvement. [16]

Bustoros et al identified the following as independent predictors of progression from asymptomatic to overt Waldenström macroglobulinemia [17] :

  • Immunoglobulin M ≥4500 mg/dL
  • Bone marrow lymphoplasmacytic infiltration ≥70%
  • Beta2-microglobulin ≥4.0 mg/dL
  • Serum albumin ≤3.5 g/dL 

Kastritis et al have developed and validated an updated international prognostic score system for Waldenström macroglobulinemia. [18] 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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