NK/T Cell Lymphoma: A Tertiary Centre Experience

Abstract

Extranodal NK/T-cell lymphoma (ENKTL), nasal type, is a rare type of non-Hodgkin lymphoma that is commonly seen in East Asian countries and is associated with Epstein-Barr virus infection. This is a retrospective study where we describe nine cases of ENKTL; nasal type diagnosed and treated at our center over a period of 2 years. These cases were analyzed retrospectively for clinical presentation (age, sex, site of involvement), immunophenotype, treatment, response and toxicity profile. Sino-nasal symptoms (blocked nose, headache, epistaxis, regurgitation) were the most common presenting complaints (n = 8). Almost 67 % (n = 6/9) of the cases were referred from Bhutan. Necrosis and angiocentricity were the commonest histological features. Depending on the stage of the disease patient were initially treated with chemotherapy (SMILE/CHOP) and Radiotherapy (IFRT). We describe our experience of management of ENKTL, where we observe that Bhutan could be endemic region for this rare tumour.

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…The cornerstone of any patient evaluation centers around complete history and physical examination (Table XXVI.1). Sinonasal lymphoma is no different with most patients initially being evaluated for sinonasal symptoms as their chief complaint 1787 . The most likely subtype of lymphoma varies with geography, with B‐cell being more common in Europe and North America and ENKTL arising more frequently in Asia and South America 1774,1788,1789 …”

“…Regardless of the location of consultation, history should focus on the nature of the symptoms and duration. A retrospective series examining the most common presenting symptoms suggests that most patients present with nonspecific complaints, most often sinonasal symptoms that can be easily confused for CRS 1785,1787,1790–1792 . Interestingly, there has been report of the impact of comorbid CRS on ENKTL stage finding that the duration of CRS symptoms correlated with the stage of disease at the time of diagnosis 1793 .…”

“…Alternatively, RT and cisplatin followed by three cycles of VIPD (etoposide, ifosfamide, cisplatin, and dexamethasone) could be considered. Other options if concurrent treatment is not pursued are the “sandwich” regimen (pegaspargase, gemcitabine, oxaliplatin [P‐GEMOX] × two cycles followed by RT, and then another two to three rounds of P‐GEMOX) or sequential regimen (for stage I and II, modified steroid [dexamethasone], methotrexate, ifosfamide, pegaspargase, and etoposide [SMILE] two to four cycles followed by RT) 1787,1847 . There are no robust studies for guidance on differential survival benefits for a particular patient and no recommendation can be made on differential survival by treatment regimen.…”