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      Patterns of survival in lymphoplasmacytic lymphoma/Waldenström macroglobulinemia: a population-based study of 1,555 patients diagnosed in Sweden from 1980 to 2005.

      American Journal of Hematology

      mortality, Adult, drug therapy, diagnosis, Waldenstrom Macroglobulinemia, Time Factors, epidemiology, Sweden, Survival Rate, Retrospective Studies, Middle Aged, Male, Humans, Female, Disease-Free Survival, administration & dosage, Antineoplastic Agents, Aged, 80 and over, Aged, Age Factors

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          Clinical management of lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinemia (WM) has changed considerably over recent years, reflected in the use of new therapeutic agents (purine analogs, monoclonal antibodies, thalidomide- and bortezomib-based therapies). No population-based studies and few randomized trials have been performed to assess survival in newly diagnosed LPL/WM. We performed a large population-based study in Sweden including 1,555 LPL/WM patients diagnosed from 1980 to 2005. Relative survival ratios (RSRs) and excess mortality rate ratios (EMRR) were computed as measures of survival. Survival of LPL/WM patients has improved significantly (P = 0.007) over time with 5-year RSR = 0.57 (95% confidence interval [CI] 0.46-0.68), 0.65 (0.57-0.73), 0.74 (0.68-0.80), 0.72 (0.66-0.77), and 0.78 (0.71-0.85) for patients diagnosed during the calendar periods 1980-1985, 1986-1990, 1991-1995, 1996-2000, and 2001-2005, respectively. Improvement in 1- and 5-year relative survival was found in all age groups and for LPL and WM separately. Patients with WM had lower excess mortality compared to LPL (EMRR = 0.38; 95% CI 0.30-0.48). Older age at diagnosis was associated with a poorer survival (P < 0.001). Taken together, we found a significant improvement in survival in LPL/WM over time. Despite this progress, new effective agents with a more favourable toxicity profile are needed to further improve survival in LPL/WM, especially in the elderly. Copyright © 2012 Wiley Periodicals, Inc.

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