Open AccessExtranodal NK-T Cell Lymphoma, Nasal Type: Retrospective Analysis of Real-World Data
Author(s) -
Sneha Bothra,
Pragya Bhandari,
Narendra Agrawal,
Narender Tejwani,
Rayaz Ahmed,
Vishvdeep Khushoo,
Sumeet Mirgh
Publication year - 2019
Publication title -
indian journal of hematology and blood transfusion/indian journal of hematology and blood transfusion
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.213
H-Index - 15
eISSN - 0974-0449
pISSN - 0971-4502
DOI - 10.1007/s12288-019-01195-6
Subject(s) - medicine , lymphoma , regimen , stage (stratigraphy) , t cell lymphoma , gastroenterology , hematology , malignancy , retrospective cohort study , aggressive lymphoma , chemotherapy , oncology , rituximab , paleontology , biology
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.