L Magnano1, O Balagué2, I Dlouhy3, J Rovira3, K Karube4, M Pinyol5, A Rivas-Delgado3, D Costa6, A Martínez-Trillos3, B González-Farre2, A Martínez-Pozo2, E Giné3, D Colomer6, J Delgado3, N Villamor6, E Campo6, A López-Guillermo7. 1. Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona;; Hematopathology Unit, Department of Pathology, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona;; CIBERONC, Barcelona. 2. CIBERONC, Barcelona;; Department of Pathology, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain. 3. Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona;; CIBERONC, Barcelona. 4. Department of Pathology and Cell Biology, Graduate School of Medicine, University of the Ryukyus;; Faculty of Medicine, University of the Ryukyus, Japan. 5. CIBERONC, Barcelona;; Genomics Unit, IDIBAPS, Barcelona, Spain. 6. Hematopathology Unit, Department of Pathology, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona;; CIBERONC, Barcelona. 7. Department of Hematology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona;; CIBERONC, Barcelona;. Electronic address: alopezg@clinic.ub.es.
Abstract
BACKGROUND: The co-existence at diagnosis of follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) components (FL/DLBCL) has been considered a transformed lymphoma and accordingly treated although clinicobiological information on these patients is scarce. The aim of this study was to analyze the initial features and outcome of FL/DLBCL patients in the rituximab era. PATIENTS AND METHODS: All patients consecutively diagnosed at a single institution with FL/DLBCL (n = 40), as well as those with pure FL (n = 328) or de novo DLBCL (n = 510) as controls. RESULTS: The proportion of the DLBCL component was highly variable (median 50%). In 29 FL/DLBCL cases analyzed, the cell of origin was GCB in 86%, ABC in 10% and unclassifiable in 4%. NOTCH1-2 was mutated in 10% of these cases. The proportion of DLBCL component did not impact on overall survival (OS). Regarding initial characteristics, patients with FL/DLBCL were closer to FL in terms of primary nodal origin, good performance status and advanced stage, whereas the other features were intermediate between FL and DLBCL. FL/DLBCL patients were treated as DLBCL with no further intensification. Complete response and primary refractory rates were 65% and 20%, respectively, with these figures being similar to DLBCL and worse than FL. Progression-free survival and OS were intermediate between FL and DLBCL (5-year OS: 85%, 73% and 63% for FL, FL/DLBCL and DLBCL, respectively). FL/DLBCL histology did not reach independent prognostic value for OS in the multivariate analyses. CONCLUSIONS: The outcome of FL/DLBCL patients is not worse than that of de novo DLBCL. These cases should be treated with immunochemotherapy as DLBCL, but intensification with ASCT may not be necessary. The biological insights of FL/DLBCL warrants further genetic and molecular studies.
BACKGROUND: The co-existence at diagnosis of follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) components (FL/DLBCL) has been considered a transformed lymphoma and accordingly treated although clinicobiological information on these patients is scarce. The aim of this study was to analyze the initial features and outcome of FL/DLBCL patients in the rituximab era. PATIENTS AND METHODS: All patients consecutively diagnosed at a single institution with FL/DLBCL (n = 40), as well as those with pure FL (n = 328) or de novo DLBCL (n = 510) as controls. RESULTS: The proportion of the DLBCL component was highly variable (median 50%). In 29 FL/DLBCL cases analyzed, the cell of origin was GCB in 86%, ABC in 10% and unclassifiable in 4%. NOTCH1-2 was mutated in 10% of these cases. The proportion of DLBCL component did not impact on overall survival (OS). Regarding initial characteristics, patients with FL/DLBCL were closer to FL in terms of primary nodal origin, good performance status and advanced stage, whereas the other features were intermediate between FL and DLBCL. FL/DLBCL patients were treated as DLBCL with no further intensification. Complete response and primary refractory rates were 65% and 20%, respectively, with these figures being similar to DLBCL and worse than FL. Progression-free survival and OS were intermediate between FL and DLBCL (5-year OS: 85%, 73% and 63% for FL, FL/DLBCL and DLBCL, respectively). FL/DLBCL histology did not reach independent prognostic value for OS in the multivariate analyses. CONCLUSIONS: The outcome of FL/DLBCL patients is not worse than that of de novo DLBCL. These cases should be treated with immunochemotherapy as DLBCL, but intensification with ASCT may not be necessary. The biological insights of FL/DLBCL warrants further genetic and molecular studies.
Authors: Yucai Wang; Brian K Link; Thomas E Witzig; Matthew J Maurer; Cristine Allmer; Rebecca L King; Andrew L Feldman; Thomas M Habermann; Stephen M Ansell; Susan L Slager; James R Cerhan; Grzegorz S Nowakowski Journal: Blood Date: 2019-10-17 Impact factor: 22.113
Authors: Heike Horn; Christian Kohler; Raphael Witzig; Markus Kreuz; Ellen Leich; Wolfram Klapper; Michael Hummel; Markus Loeffler; Lorenz Trümper; Rainer Spang; Andreas Rosenwald; German Ott Journal: Haematologica Date: 2018-03-22 Impact factor: 9.941