淋巴瘤
淋巴细胞增多症
爱泼斯坦-巴尔病毒
病毒
胃肠病学
国际预后指标
T细胞淋巴瘤
自然杀伤细胞
T细胞受体
医学
基因重排
免疫学
疾病
内科学
病理
生物
T细胞
弥漫性大B细胞淋巴瘤
基因
免疫系统
细胞毒性T细胞
生物化学
体外
作者
Leiming Hu,Katsuyoshi Takata,Tomoko Miyata‐Takata,Naoko Asano,Emiko Takahashi,Katsuya Furukawa,Hiroaki Miyoshi,Akira Satou,Kei Kohno,Hiroshi Kosugi,Tomohiro Kinoshita,Yoshiki Hirooka,Hidemi Goto,Shigeo Nakamura,Seiichi Kato
出处
期刊:Histopathology
[Wiley]
日期:2017-03-28
卷期号:70 (7): 1052-1063
被引量:13
摘要
Aims Epstein–Barr virus‐positive (EBV + ) intestinal T/natural killer ( NK ) cell lymphoma ( ITNKL ) is an uncommon tumour with an extremely aggressive clinical behaviour. However, the clinicopathological characteristics of this tumour, including T cell receptor ( TCR ) phenotype and the patient's background, remain unknown. The aim of this study was to elucidate the detailed clinicopathological profile of EBV + ITNKL . Methods and results We enrolled 12 patients with EBV + ITNKL without nasal involvement into the study. All patients were characterized by involvement of the small intestine with concurrent lesions of the large intestine in two patients. Seven patients (58%) had Lugano stages IIE/IV disease and eight (67%) were categorized as high–intermediate/high‐risk according to the Prognostic Index for PTCL ( PIT ). Three patients (25%) with an age of onset of less than 50 years had chronic active EBV infection ( CAEBV ). Five CD56‐positive patients (42%) had a poorer prognosis than those without CD56 expression ( P = 0.008). NK cell‐type lymphoma defined by the absence of any TCR expression or clonal TCR ‐γ rearrangement was found in six patients (50%). Interestingly, EBV + intra‐epithelial lymphocytosis was observed in one case with a background of CAEBV . Conclusions This study is the first to shed light on the significant heterogeneity of EBV + ITNKL and its relationship with CAEBV , especially in patients younger than 50 years of age. These observations will provide a guide for diagnostic and therapeutic approaches in routine practice.
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