Extranodal natural killer (NK)/T-cell lymphoma, nasal-type is a highly aggressive disease more frequent in Asia than in Western countries. There is no consensus treatment. The outcome depends on disease stage. Localized NK/T-cell lymphomas often respond to radiotherapy. In contrast, patients who have extensive disease or who relapse after radiotherapy have a very poor prognosis. Overall, long-term survival in these lymphomas tends to be inferior to that for other aggressive lymphomas. This review focuses on the new management modalities in light of advances in risk stratification, patient monitoring and treatment strategies.
Many parameters have been reported to correlate with prognosis and new staging systems have been elaborated. Detecting Epstein–Barr virus (EBV) in the bone marrow is important for staging and measuring EBV DNA in the serum improved monitoring response to therapy. Radiation modalities have been precised and new strategies combining radiation and chemotherapy have been proposed for patients with localized disease. The particular efficacy of L-asparaginase in this disease has been confirmed and L-asparaginase-based regimens have been studied in prospective trials for patients with refractory, relapsing or disseminated disease with good results. Laboratory studies may point the way toward new therapeutic approaches.
Early-stage disease is treated by involved-field radiotherapy with adjuvant chemotherapy. L-Asparaginase-containing regimens are the mainstay of treatment for advanced or disseminated disease. The role of targeted therapies, autologous and allogeneic haematopoietic stem cell transplantation is yet to be clearly defined.
aCentre Hospitalier Universitaire (CHU) Limoges, Université et Centre National de la Recherche Scientifique (CNRS), Unité Mixte de Recherche (UMR) 6101, Limoges
bHôpital Necker, Paris, service d’Hématologie Clinique, Université Paris Descartes, CNRS UMR 8147, Centre de Référence des Mastocytoses, Paris, France
Correspondence to Arnaud Jaccard, Service d’Hématologie et de Thérapie Cellulaire, 1 av, ML-King, CHU Limoges, France E-mail: firstname.lastname@example.org