Abstract

Objective To improve the understanding of clinical and pathological features of extranodal NK/T cell lymphoma,nasal type (NKTL) with poor prognosis and provide experiential references via a retrospective analysis.Methods 117 NKTL cases in a single center were retrospectively analyzed of their pathologic diagnoses and clinical manifestations,especially primary sites.Pathological examinations were mainly depended on morphology,immunohistochemisty for immunophenotype,and in situ hybridization for Epstein-Barr virus (EBV) encoded small RNA (EBER).Polymerase chain reaction (PCR) for whole-blood EBV DNA and T-cell receptor (TCR) gene rearrangement was performed.Chemotherapy and radiotherapy were the main treatments.International prognostic index,Ki-67,2 years overall survival (OS) rate and progressive free survival (PFS) rate according to the clinical characteristics were included in the univariable analysis.Results The positive rate for CD3 was 90.6 %,94.0 % for CD56,92.9 % for CD45RO,97.9 % for TIA,97.7 % for Granzyme B and 100.0 % for EBER,respectively.The median age was 43.2 (14-77) years old.The primary nasal NKTL was 95 cases (81.2 %) and their average Ki-67 was (48.3±2.6) %.Other primary extranodal NKTL was 22 cases (18.8 %),including primary posterior pharyngeal wall,tongue,tonsil,laryngeal,lymph nodes,skin,liver,intestinal,central nervous system and testis.Patients with primary liver or intestinal NKTL or Ki-67 greater than 80 % died in the first year.Patients with primary liver and intestinal NKTL had higher Ki-67 than patients with primary nasal.Compared to the 2 years OS rate 60.0 % and PFS rate 36.0 % of patients with Ki-67 from 60 % to 80 %,the OS rate (86.3 %) and PFS rate (57.5 %) of patients with Ki-67 from 30 % to 60 % were higher (P =0.047,0.070),and the OS rate (100.0 %) and PFS rate (78.0 %) of patients with Ki-67 less than 30 % were also higher (P =0.01,0.02).2 years OS rate of 8 CD56 negative patients whose T cell rearrangement was positive and primary sites were nasal was higher than that of 109 CD56 positive patients (P =0.03,0.02).Among the 13 EBV DNA samples detected,8 samples were normal and OS rate was 100.0 %.5 samples had more than 6.1×107 copies/ml and OS rate was 60.0 %.Conclusion It is implied that Ki-67,CD56,EBER,EBV-DNA and primary site are related with the prognosis of NKTL. Key words: Lymphoma, extranodal NK/T cell; Pathology, clinical; EB virus

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