Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type

Abstract

The World Health Organization (WHO) classification recognizes 2 main categories of natural killer (NK) cell-derived neoplasms, namely, extranodal NK/T-cell lymphoma, nasal type, and aggressive NK-cell leukaemia. Extranodal nasal NK/T-cell lymphoma is more frequent in the Far East and Latin America. Histopathological and immunophenotypical hallmarks include angiocentricity, angiodestruction, expression of cytoplasmic CD3 epsilon (ε), CD56, and cytotoxic molecules and evidence of Epstein-Barr virus (EBV) infection. Early stage disease, in particular for localized lesion in the nasal region, is treated with chemotherapy and involved-field radiotherapy. On the other hand, multiagent chemotherapy is the mainstay of treatment for advanced or disseminated disease. L-asparaginase-containing regimens have shown promise in treating this condition. The role of autologous hematopoietic stem cell transplantation is yet to be clearly defined. Allogeneic hematopoietic stem cell transplantation, with the putative graft-versus-lymphoma effect, offers a potentially curative option in patients with advanced disease.

Racial patterns of extranodal natural killer/T‐cell lymphoma, nasal type, in California: a population‐based study

“…For this reason, combined chemotherapy and radiotherapy has become the preferred treatment in many institutions for patients who can tolerate systemic therapy (Au, 2010). Recent Phase II studies of combined modality treatment for localized ENKTL have yielded excellent results with an overall response rate of 83%, complete response rate of 73% and 3-year OS of 80% Yamaguchi et al, 2009;Gill et al, 2010). Supporting the use of combined modality therapy, and similar to a previous report (Au et al, 2009), our study found that initial treatment with chemotherapy plus radiotherapy, but not radiotherapy alone, was associated with superior OS in local/regional disease.…”

Meta Analysis of Treatment for Stage IE~IIE Extranodal Natural Killer /T Cell Lymphomas in China

“…Its morbidity is consistent with the rate of EB virus infection in the geographic distribution. Research in recent years has demonstrated that CD3 and CD56 present in the cytoplasm of theses tumor cells, which simulteneously express cytotoxic granule proteins, such as TIA-1 and EB virus antigen (Steven H et al, 2008;Au WY et al, 2009;Gill H et al, 2010;Gualco G et al, 2011;Shaoying Li et al, 2013) . ENK/TCL has poor prognosis and is influenced by many factors, including age, LDH value, the titer of Epstein-Barr virus (EBV) DNA, absolute lymphocyte count and the positive expression of CD30 (Au et al, 2004;Lee et al, 2005;Hong et al, 2012;Li et al, 2013) etc.…”

“…Therefore, comprehensive and detailed evaluation of patients should be done before the therapy, so as to apply individualized treatment and benefit the patients. With respect to the scheme of combine therapy, large number of studies Gill et al, 2010;Zheng et al, 2012;Kang et al, 2012;ect) has reported that the radioactivity of 50Gy and1.8-2Gy/time are optimal for the patients, who poorly response to CHOP chemotherapy because these patients are not sensitive to anthracycline-based chemotherapy scheme duo to multidrug resistance induced by high expression level of P-glycoprotein . Whether etoposide or L-asparaginase-based chemotherapy that can overcome multidrug resistance mediated by P-glycoprotein should be applied still requires further research (Yong et al, 2009;.…”

Survival benefit of radiotherapy on patients with early-stage extranodal nasal-type natural killer/T-cell lymphoma: an analysis of the surveillance epidemiology and end results database

“…ENKTL occurs predominantly in the nasal cavity and nasal side area and the disease is highly aggressive, is not sensitive to chemotherapy and has poor prognosis. The 5-year overall survival (OS) ranges from 40% to 90% for early-stage patients [3][4][5][6][7].…”