Durot, E;
Kanagaratnam, L;
Zanwar, S;
Kastritis, E;
D'Sa, S;
Garcia-Sanz, R;
Tomowiak, C;
... Castillo, JJ; + view all
(2020)
A prognostic index predicting survival in transformed Waldenström macroglobulinemia.
Haematologica
, 106
(11)
10.3324/haematol.2020.262899.
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Abstract
Histological transformation into diffuse large B-cell lymphoma is a rare complication in patients with Waldenström macroglobulinemia (WM) usually associated with a poor prognosis. The objective of this study was to develop and validate a prognostic index for survival in transformed WM patients. Through this multicenter, international collaborative effort, 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 end-point. For external validation, a data set 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 LDH (2 points), platelet count < 100 x 109/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 (HR) = 3.4) and high-risk (4 points, 17%, HR = 7.5). Two-year survival rates were 81%, 47%, and 21%, respectively (P < 0.0001). This model appeared to be a better discriminant than the International Prognostic Index (IPI) and the revised IPI (R-IPI). We validated this 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.
| Type: | Article |
|---|---|
| Title: | A prognostic index predicting survival in transformed Waldenström macroglobulinemia |
| Location: | Italy |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3324/haematol.2020.262899 |
| Publisher version: | https://doi.org/10.3324/haematol.2020.262899 |
| Language: | English |
| Additional information: | This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
| Keywords: | Humans, Lymphoma, Large B-Cell, Diffuse, Prognosis, Proportional Hazards Models, Survival Rate, Waldenstrom Macroglobulinemia |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10132696 |
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