Data on clinical features observed in patients with Waldenstrom's macroglobulinemia (WM) during follow-up remain limited. Therefore, we evaluated 860 follow-up procedures in 101 patients. Median age was 66 years and 5-year overall survival 72%, with a median follow-up of 36 months in surviving patients. Sixteen patients presented at diagnosis with two or three cytopenias lasting for at least 3 months (multiple cytopenias [MC]), and MC improved after treatment in eight patients, 4 to 18 months later. MC was observed during at least 6 consecutive months in 23 other patients, 2 to 73 months (median, 24) after diagnosis. MC occurred off-therapy in 12 patients, and on-therapy in 11. Regression occurred in three of the former patients, and in seven of the latter (6 to 24 months after completion of treatment; median, 7). Finally, the 4-year estimated cause-specific cumulative incidence was 40% in the 101 patients. A second malignancy was observed in 11 patients, histological transformation in three, and rapid rise of M-component in only six patients. In conclusion, the present analysis pointed out a high incidence of long lasting MC during the evolution of WM, and a low frequency of rapid rise of M component.
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