A PROGNOSTIC INDEX PREDICTING SURVIVAL IN TRANSFORMED WALDENSTRöM MACROGLOBULINEMIA

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

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Histological transformation into diffuse large B-cell lymphoma is a rare complication in patients with Waldenström macroglobulinemia (WM) and is usually associated with a poor prognosis.The objective of this study was to develop and validate a prognostic index for survival of patients with transformed WM.Through this multicenter, international collaborative effort, arrethe bush balm we developed a scoring system based on data from 133 patients with transformed WM who were evaluated between 1995 and 2016 (training cohort).Univariate and multivariate analyses were used to propose a prognostic index with 2-year survival after transformation as an endpoint.

For external validation, a dataset of 67 patients was used to evaluate the performance of the model (validation cohort).By multivariate analysis, three adverse covariates were identified as independent predictors of 2-year survival after transformation: elevated serum lactate dehydrogenase (2 points), platelet count <100x109/L (1 point) and any previous treatment for WM (1 point).Three risk groups were defined: low-risk (0-1 point, 24% of patients), intermediate-risk (2-3 points, 59%; hazard ratio = 3.4) and high-risk (4 points, 17%; hazard ratio = 7.

5).Two-year survival rates were 81%, 47%, and 21%, respectively (P<0.0001).This model appeared to be a better discriminant than either the International Prognostic Index or the revised International Prognostic Index.

We validated this 730 sunken lake road model in an independent cohort.This easy-to-compute scoring index is a robust tool that may allow identification of groups of transformed WM patients with different outcomes and could be used for improving the development of risk-adapted treatment strategies.

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