Improved outcomes for extranodal natural killer T-cell lymphoma
Menée à partir de données portant sur 166 patients atteints d'un lymphome extra-ganglionnaire à cellules T/NK, cette étude internationale de cohorte prospective analyse les facteurs cliniques et thérapeutiques associés à la survie globale à 5 ans (durée médiane de suivi : 44 mois)
Although understanding of the heterogeneous landscape of natural killer (NK)-cell malignancies remains limited, extranodal NK T-cell lymphoma (ENKTL) stands out globally as a distinct disease entity; its clinical presentation is often disfiguring, and it shows chemoresistance and universal association with the Epstein-Barr virus (EBV). As for many other types of T-cell and NK-cell neoplasms, especially those associated with Epstein-Barr virus, the incidence and prevalence of ENKTL are substantially higher in east Asia and South America compared with in Europe and North America. Studies primarily done in Asian centres have shaped the current approach to ENKTL, in particular highlighting the importance of radiation therapy in early-stage disease, the unique efficacy of L-asparaginase, and the importance of plasma Epstein-Barr virus monitoring in prognosis and disease surveillance. However, insights from other T-cell and NK-cell neoplasms indicate that both the genetic landscape and the natural history of these neoplasms can differ markedly according to race, ethnicity, and geography.
ENKTL in European and North American patients versus Asian patients varies in clinical presentation, age at diagnosis, frequency of antecedent lymphoproliferative disorders, and survival outcome. Thus, the inclusion of geographically and ethnically diverse populations is essential in large outcome studies that shape practice.
The Lancet Haematology , commentaire, 2019