TY - JOUR AU - Clémentine Sarkozy, AU - Thierry Molina, AU - Hervé Ghesquières, AU - Anne-Sophie Michallet, AU - Jehan Dupuis, AU - Diane Damotte, AU - Franck Morsschauser, AU - Marie Parrens, AU - Laurent Martin, AU - Peggy Dartigues, AU - Aspasia Stamatoullas, AU - Pierre Hirsch, AU - Bettina Fabiani, AU - Krimo Bouabdallah, AU - Maria Gomes da Silva, AU - Marie Maerevoet, AU - Camille Laurent, AU - Bertrand Coiffier, AU - Gilles Salles, AU - Alexandra Traverse-Glehen, PY - 2016/12/31 Y2 - 2024/03/29 TI - Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association JF - Haematologica JA - haematol VL - 102 IS - 1 SE - Articles DO - 10.3324/haematol.2016.152256 UR - https://haematologica.org/article/view/7933 SP - 150-159 AB - Mediastinal gray zone lymphoma, B-cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma, have not been well described in the literature. We report the clinical characteristics and outcomes of a large retrospective series of 99 cases centrally reviewed by a panel of hematopathologists, with a consensus established for the diagnosis. Cases were defined as classical Hodgkin lymphoma-like morphology (64.6%) with primary mediastinal B-cell lymphoma immunophenotype, primary mediastinal B-cell lymphoma-like morphology (30.3%) with classical Hodgkin lymphoma or composite (5.1%) (synchronous occurrence of classical Hodgkin lymphoma and primary mediastinal B-cell lymphoma). The median age was 32 years (13–83 years); 55% were women. Thirteen of 81 evaluable cases (16%) were Epstein-Barr virus-positive. Twenty-eight percent of patients presented primary refractory disease (progression under first-line treatment or relapse within one year). The 3-year event-free and overall survival rates were 63% and 80%, respectively. Patients treated with a standard regimen (RCHOP/ABVD) had worse event-free survival (P=0.003) and overall survival (P=0.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escalated BEACOPP). Rituximab added to chemotherapy was not associated with better event-free survival (P=0.55) or overall survival (P=0.88). Radiotherapy for patients in complete remission had no impact on event-free survival. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter event-free survival and overall survival, respectively. In conclusion, this report describes the largest series of mediastinal gray zone lymphoma. Our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease. ER -