BACKGROUND: B-cell lymphoma, Unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, for convenience referred to here as unclassifiable B-cell lymphoma, is a category in the 2008 World Health Organization system used for a group of histologically aggressive neoplasms that are difficult to classify definitively. Currently, there is no established standard therapy for these neoplasms. METHODS: The authors assessed MYC status and correlated it with treatment response and outcome in a group of 52 patients with unclassifiable B-cell lymphoma treated with either a standard DLBCL regimen (R-CHOP [rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone-related therapy]) or more intensive regimens, such as R-hyper-CVAD (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine). The regimens were selected by the treating clinicians based on the overall clinical and pathological findings. RESULTS: Thirty (58%) unclassifiable B-cell lymphomas had MYC abnormalities (MYC(+) ) including 27 with rearrangement, 2 with amplification, and 1 with both. The MYC(+) and MYC(-) groups were similar in their age distribution and International Prognostic Index scores. Progression-free survival of patients with MYC(+) unclassifiable B-cell lymphoma treated initially with R-CHOP was significantly worse than patients treated with R-hyper-CVAD (P = .0358). In contrast, for the MYC(-) unclassifiable B-cell lymphoma group, some patients responded to R-CHOP, and others were refractory to R-hyper-CVAD. CONCLUSIONS: MYC aberrations are common in unclassifiable B-cell lymphoma. The presence of MYC aberrations identifies a patient subset that requires more aggressive therapy than R-CHOP. In contrast, MYC(-) unclassifiable B-cell lymphoma patients responded variably to either R-CHOP or aggressive therapy, and the latter showed no survival advantage.
BACKGROUND:B-cell lymphoma, Unclassifiable with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, for convenience referred to here as unclassifiable B-cell lymphoma, is a category in the 2008 World Health Organization system used for a group of histologically aggressive neoplasms that are difficult to classify definitively. Currently, there is no established standard therapy for these neoplasms. METHODS: The authors assessed MYC status and correlated it with treatment response and outcome in a group of 52 patients with unclassifiable B-cell lymphoma treated with either a standard DLBCL regimen (R-CHOP [rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone-related therapy]) or more intensive regimens, such as R-hyper-CVAD (rituximab plus hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with high-dose methotrexate and cytarabine). The regimens were selected by the treating clinicians based on the overall clinical and pathological findings. RESULTS: Thirty (58%) unclassifiable B-cell lymphomas had MYC abnormalities (MYC(+) ) including 27 with rearrangement, 2 with amplification, and 1 with both. The MYC(+) and MYC(-) groups were similar in their age distribution and International Prognostic Index scores. Progression-free survival of patients with MYC(+) unclassifiable B-cell lymphoma treated initially with R-CHOP was significantly worse than patients treated with R-hyper-CVAD (P = .0358). In contrast, for the MYC(-) unclassifiable B-cell lymphoma group, some patients responded to R-CHOP, and others were refractory to R-hyper-CVAD. CONCLUSIONS:MYC aberrations are common in unclassifiable B-cell lymphoma. The presence of MYC aberrations identifies a patient subset that requires more aggressive therapy than R-CHOP. In contrast, MYC(-) unclassifiable B-cell lymphomapatients responded variably to either R-CHOP or aggressive therapy, and the latter showed no survival advantage.
Authors: Sietse M Aukema; Markus Kreuz; Christian W Kohler; Maciej Rosolowski; Dirk Hasenclever; Michael Hummel; Ralf Küppers; Dido Lenze; German Ott; Christiane Pott; Julia Richter; Andreas Rosenwald; Monika Szczepanowski; Carsten Schwaenen; Harald Stein; Heiko Trautmann; Swen Wessendorf; Lorenz Trümper; Markus Loeffler; Rainer Spang; Philip M Kluin; Wolfram Klapper; Reiner Siebert Journal: Haematologica Date: 2013-10-31 Impact factor: 9.941
Authors: Gabriella Aquino; Laura Marra; Monica Cantile; Annarosaria De Chiara; Giuseppina Liguori; Maria Pia Curcio; Rocco Sabatino; Giuseppe Pannone; Antonio Pinto; Gerardo Botti; Renato Franco Journal: Infect Agent Cancer Date: 2013-09-30 Impact factor: 2.965
Authors: Daniel J Landsburg; Marissa K Falkiewicz; Adam M Petrich; Benjamin A Chu; Amir Behdad; Shaoying Li; L Jeffrey Medeiros; Ryan D Cassaday; Nishitha M Reddy; Martin A Bast; Julie M Vose; Kimberly R Kruczek; Scott E Smith; Priyank Patel; Francisco Hernandez-Ilizaliturri; Reem Karmali; Saurabh Rajguru; David T Yang; Joseph J Maly; Kristie A Blum; Weiqiang Zhao; Charles Vanslambrouck; Chadi Nabhan Journal: Br J Haematol Date: 2016-07-29 Impact factor: 6.998
Authors: Jonathon B Cohen; Susan M Geyer; Gerard Lozanski; Weiqiang Zhao; Nyla A Heerema; Nathan C Hall; Veena A Nagar; Jessica A Hemminger; Jeffrey A Jones; Pierluigi Porcu; Beth A Christian; Robert A Baiocchi; Kami J Maddocks; Joseph M Flynn; Steven M Devine; Kristie A Blum Journal: Cancer Date: 2014-02-27 Impact factor: 6.860