De-escalating Chemotherapy for Advanced Extranodal Natural Killer/T-Cell Lymphoma: A Step Toward Improved Treatment of a Rare Virus-Associated Cancer
Mené en Chine sur 87 patients atteints d'un lymphome extra-ganglionnaire à cellules T/NK de stade avancé et récemment diagnostiqué, cet essai randomisé compare l'efficacité, du point de vue de la survie sans progression, et la toxicité d'une chimiothérapie de type DDGP (dexaméthasone, cisplatine, gemcitabine et pégaspargase) et d'une chimiothérapie de type SMILE (dexaméthasone, méthotrexate, ifosfamide, L-asparaginase et étoposide)
Extranodal natural killer/T-cell lymphoma (ENKL), nasal type (ENKL-NT) is a rare, aggressive Epstein-Barr virus (EBV)-associated cancer most frequently occurring in Asian and Central and South American populations. A pooled registry analysis suggests ENKL-NT comprises 6.6% of lymphoma cases in East and Southeast Asia, 2.9% in Latin America, and 0.2% in the rest of the world. Within the United States, ENKL-NT is most common in people with Asian or Latin American ancestry. All patients with ENKL-NT are infected with EBV with latency type 2 pattern of viral gene expression. Lack of detection of EBV-encoded small RNA in situ hybridization (EBER) in the tumor rules out a diagnosis of ENKL-NT. Epstein-Barr virus plays a large role in lymphomagenesis of ENKL-NT. Sequencing of viral terminal repeats reveals that EBV is clonal, implying that infection occurs prior to the development of ENKL-NT. Patients with ENKL-NT should be distinguished from those with rarer EBV+ T-cell and natural killer–cell lymphomas such as systemic EBV+ T-cell lymphoma of childhood and nodal EBV+ peripheral T-cell lymphomas, which have distinct clinical and pathologic features. Patients with ENKL-NT may develop hemophagocytic lymphohistiocytosis, which complicates therapy and is associated with high mortality. Emerging data suggest that ENKL-NT may be associated with inherited variants in genes related to lymphocyte cytotoxicity, including LYST, UNC13D, PRF1, and AP3B1 in approximately one-third of cases from one series of Chinese patients.
JAMA Oncology , éditorial, 2021