Extranodal NK-T Cell Lymphoma, Nasal Type: Retrospective Analysis of Real-World Data.

Peripheral T cell lymphomas constitute nearly 15% of all cases on non-Hodgkin lymphoma. Of these, NK-T cell lymphoma nasal type is a rare and aggressive form. We present our experience of 16 patients of NK/T cell lymphoma which constituted approximately 1% of all lymphoma (N = 1590) cases treated at our center. Male to female ratio was 4.3:1. Median age of presentation was 42 years. Early Stage patients (n = 11) were treated with DeVIC regimen (n = 10) and SMILE (n = 1) chemotherapy and RT to all the patients. Advanced stage patients were treated with SMILE regimen (n = 4) and ICE and local RT (n = 1) with one treatment related mortality. The presence of B-symptoms adversely affected survival. The estimated median PFS and OS were 39 and 49 months respectively. Overall survival was not reached in Limited Stage patients (stage 1 and 2) and 8 months in patients with advanced stage (stage IV) (p = 0.001). According to the new CSWOG staging (retrospectively applied), comparing the Limited versus Extensive Stage, the earlier group has a significantly better estimated PFS (p = 0.020) and OS (p = 0.007). ENKTL is a rare malignancy with aggressive course. B-symptoms portend a poor prognosis to patients with this aggressive lymphoma. The new staging system helps estimate survival better. © Indian Society of Hematology and Blood Transfusion 2019.