Symptomatic Waldenstrom’s Macroglobulinemia (WM) in Young Patients: Disease Characteristics and Outcome.

Abstract Introduction: WM is a disease of the elderly with a median age of 70 years in most series. Advanced age is recognized as an adverse prognostic feature and age > 65 years has been included among the 5 adverse covariates in the recently reported International prognostic Scoring System for WM (Morel et al, ASH 2006). There is little information regarding the incidence of disease features and outcomes after treatment of young patients (≤ 50 years of age) with symptomatic WM. Patients and methods: Our database includes 220 patients with previously untreated, symptomatic WM. These patients were separated in two groups according to age ≤ 50 years and >50 years at the time of initiation of treatment. Clinical and laboratory characteristics, response to treatment, overall survival (OS) and disease-specific survival (DSS) were compared among the two groups. Results: Twenty-two patients (10%) were ≤ 50 years of age at the time of initiation of treatment. The incidence of clinical and laboratory features was similar among young and older patients: gender (p=0.3), B-symptoms (p=0.3), splenomegaly (p=0.9), lymphadenopathy (p=0.1), anemia (p=0.3), median serum IgM level (p=0.6), hyperviscosity syndrome (p=0.7), elevated β2-microglobulin (p=0.3) and IgM related disorders (p=0.6). primary treatment consisted of chlorambucil, nucleoside analogs or rituximab-based regimens with equal distribution among young and older patients. At least partial response was observed in 73% of young patients and in 57% of older patients (p=0.16). The median OS was not reached in young patients and it was 106 months in older patients (p=0.07). The median DSS was not reached in young patients and it was 116 months in older patients (p=0.2). Conclusion: 10% of patients with WM are ≤50 years at the time of treatment initiation. Their clinical and laboratory features and response to treatment are similar to those of older patients. Overall survival tend to be longer in young patients, but when deaths unrelated to WM or complications of treatment are excluded, survival times among young and older patients are similar.