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. 2012 May 10;119(19):4462-6.
doi: 10.1182/blood-2011-10-384768. Epub 2012 Mar 26.

Progression in smoldering Waldenstrom macroglobulinemia: long-term results

Robert A Kyle  1 , Joanne T Benson, Dirk R Larson, Terry M Therneau, Angela Dispenzieri, Shaji Kumar, L Joseph Melton 3rd, S Vincent Rajkumar
Affiliations

Progression in smoldering Waldenstrom macroglobulinemia: long-term results

Robert A Kyle et al. Blood. .

Abstract

The purpose of this study was to define the risk of progression and survival of patients with smoldering Waldenström macroglobulinemia (SWM). SWM is defined clinically as having a serum monoclonal IgM protein≥3 g/dL and/or≥10% bone marrow lymphoplasmacytic infiltration but no evidence of end-organ damage (anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly). We searched a computerized database and reviewed the medical records of all patients at Mayo Clinic who fulfilled the criteria of SWM between 1974 and 1995. During 285 cumulative person-years of follow-up of the 48 patients with SWM (median, 15.4 years), 34 (71%) progressed to symptomatic Waldenström macroglobulinemia (WM) requiring treatment, one to primary amyloidosis, and one to lymphoma (total, 75%). The cumulative probability of progression to symptomatic WM, amyloidosis, or lymphoma was 6% at 1 year, 39% at 3 years, 59% at 5 years, and 68% at 10 years. The major risk factors for progression were percentage of lymphoplasmacytic cells in the bone marrow, size of the serum M-spike, and the hemoglobin value. Patients with SWM should be followed and not treated until symptomatic WM develops. Treatment on a clinical trial for those at greatest risk of progression should be considered.

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Figures

Figure 1
Figure 1
Cumulative probability of progression to symptomatic WM requiring therapy, amyloidosis, or lymphoma in the cohort of 48 patients with SWM.
Figure 2
Figure 2
Probability of progression to symptomatic WM requiring therapy on the basis of lymphoplasmacytic bone marrow infiltration.
Figure 3
Figure 3
Probability of progression to symptomatic WM with M-spike ≥ 3 g/dL and bone marrow cells ≥ 10% and M-spike < 3 g/dL and bone marrow cells ≥ 10%.
Figure 4
Figure 4
Probability of survival of SWM in 48 patients.
Figure 5
Figure 5
Probability of survival in 34 patients with WM.
See this image and copyright information in PMC

References

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    1. Kyle RA, Treon SP, Alexanian R, et al. Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia [review]. Semin Oncol. 2003;30(2):116–120. - PubMed
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