Extranodal natural killer T-cell lymphoma—new profiling, old tricks

Abstract

Extranodal natural killer T-cell lymphomas (ENKTL) are aggressive lymphoid malignancies that present in two distinct forms: nasal and extra-nasal. 1 Au WY Weisenburger DD Intragumtornchai T et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood. 2009; 113: 3931-3937 Crossref PubMed Scopus (564) Google Scholar Several features separate it from all other non-Hodgkin lymphomas. For example, that all cases are associated with Epstein-Barr virus 2 Chan JK Yip TT Tsang WY et al. Detection of Epstein-Barr viral RNA in malignant lymphomas of the upper aerodigestive tract. Am J Surg Pathol. 1994; 18: 938-946 Crossref PubMed Scopus (171) Google Scholar suggests a unique carcinogenesis and creates an opportunity for both accurate diagnosis and sensitive molecular monitoring. Second, extranodal presentation in the upper aerodigestive tract or other sites (such as the intestine, lungs, skin, testes) and rare isolated involvement of lymph nodes 1 Au WY Weisenburger DD Intragumtornchai T et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-Cell Lymphoma Project. Blood. 2009; 113: 3931-3937 Crossref PubMed Scopus (564) Google Scholar poses specific challenges to the staging with the traditional Ann-Arbor classification system and accurate risk stratification with the International Prognostic Index. Third, chemotherapy resistance results in limited long-term disease control, and radiotherapy seems to produce durable remissions in a substantial proportion of patients with early-stage disease. 3 Li YX Yao B Jin J et al. Radiotherapy as primary treatment for stage IE and IIE nasal natural killer/T-cell lymphoma. J Clin Oncol. 2006; 24: 181-189 Crossref PubMed Scopus (302) Google Scholar Finally, extensive tissue necrosis and remodelling (and the associated inflammation and chronic infections) make interpretation of imaging studies at both diagnosis and response assessment very difficult. A prognostic index for natural killer cell lymphoma after non-anthracycline-based treatment: a multicentre, retrospective analysisPINK and PINK-E are new prognostic models that can be used to develop risk-adapted treatment approaches for patients with ENKTL being treated in the contemporary era of non-anthracycline-based therapy. Full-Text PDF