Extranodal natural killer/T cell lymphoma, nasal type (ENKL) is a rare disease, which is much more prevalent in Asia. With the advent of L-asparaginase-based regimen, the outcome of ENKL was improved obviously. Sequential chemotherapy and radiotherapy is the standard treatment for early-stage ENKL. However, the outcome of advanced-stage diseases is not satisfactory. Therefore, risk-stratification is needed for ENKL. The prognostic factors include IPI, KIPI, plasma EBV-DNA, and interim-PET/CT. However, these parameters are not validated in the era of L-asparaginase. The role of high-dose chemotherapy and heamatopoietic stem cell transplantation require further investigation.
Keywords: Lymphoma; chemotherapy; natural killer/T (NK/T); prognosis; radiotherapy; transplant.