Article révisé par les pairs
Résumé : Mediastinal grey zone lymphoma, B cell lymphomas with intermediate features between classical Hodgkin lymphoma and primary mediastinal B cell lymphoma, are not 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) with 55% of women. Thirteen out of 81 evaluable cases (16%) were EBV-positive. Twenty-eight percent of the 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 EFS (p=.003) and OS (p=.02) than those treated with a dose-intensive chemotherapy (high-dose RCHOP/escBEACOPP). Rituximab added to chemotherapy was not associated with better EFS (p=.55) or OS (p=.88). Radiotherapy for patients in CR had no impact on EFS. In multivariate prognostic analysis, ECOG-PS and anemia were the strongest factors associated with a shorter EFS and OS respectively. In conclusion, this report describes the largest series of mediastinal grey zone lymphoma; our data suggest that a dose-intensive treatment might improve the outcome of this rare and aggressive disease.

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