Περίληψη:
Histological transformation into diffuse large B-cell lymphoma is a rare
complication in patients with Waldenstrom 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, 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 <100x10(9)/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 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.
Συγγραφείς:
Durot, Eric
Kanagaratnam, Lukshe
Zanwar, Saurabh
Kastritis,
Efstathios
D'Sa, Shirley
Garcia-Sanz, Ramon
Tomowiak, Cecile
and Hivert, Benedicte
Toussaint, Elise
Protin, Caroline and
Abeykoon, Jithma P.
Guerrero-Garcia, Thomas
Itchaki, Gilad and
Vos, Josephine M.
Michallet, Anne-Sophie
Godet, Sophie and
Dupuis, Jehan
Lepretre, Stephane
Bomsztyk, Joshua
Morel,
Pierre
Leblond, Veronique
Treon, Steven P.
Dimopoulos,
Meletios A.
Kapoor, Prashant
Delmer, Alain
Castillo, Jorge
J.
