Clinicopathologic and Molecular Features of 122 Brazilian Cases of Nodal and Extranodal NK/T-Cell Lymphoma, Nasal Type, With EBV Subtyping Analysis
Clinicopathologic and Molecular Features of 122 Brazilian Cases of Nodal and Extranodal NK/T-Cell Lymphoma, Nasal Type, With EBV Subtyping Analysis
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DOI：
10.1097/pas.0b013e31821ec4b5
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发表时间：
2011-08-01
影响因子：
5.6
通讯作者：
Bacchi, Carlos E.
中科院分区：
文献类型：
--
作者：
Gualco, Gabriela;Domeny-Duarte, Pollyanna;Bacchi, Carlos E.
结外自然杀伤/T细胞淋巴瘤，鼻型(NK/TCL)在亚洲和中南美洲的一些地区较为常见，但在美国和欧洲很少见。本研究分析了122例来自巴西的NK/TCL的临床病理特征。分析97例鼻/鼻咽区和23例鼻外部位(包括6例结节)的临床特点和地理分布。在21名患者中获得了临床分期和随访信息。所有病例均携带EB病毒(EBV)，胞浆CD3和CD56的表达分别为95%和85%，且至少有一种细胞毒颗粒标志物阳性。EBV亚型的全球分布以A亚型为主，占89%，B亚型占11%。没有检测到双重感染。TCR-γTCR-基因重排7例，均位于结外。2例TCR-Gamma(+)/CD56(+)病例为EBV B亚型。不同地理区域之间存在显著差异，绝大多数B亚型EBV(86%)发生在南部和东南部地区。
Extranodal natural killer/T-cell lymphoma, nasal type (NK/TCL) is more prevalent in Asia and in some areas of South and Central America, but it is rarely seen in the United States and Europe. In this study, a series of 122 cases of NK/TCL from Brazil was analyzed with respect to clinicopathologic features. Clinical characteristics and geographic distribution were evaluated in 97 cases of nasal/nasopharyngeal region and 23 cases in extranasal sites including 6 nodal cases. Clinical staging and follow-up information was available in a subset of 21 patients. All cases harbored Epstein-Barr virus (EBV), 95% and 85% expressed cytoplasmic CD3 and CD56, respectively, and all cases were positive for at least 1 marker for cytotoxic granules. The global distribution of EBV subtypes showed predominance of strain subtype A, 89%, and subtype B, 11%. No dual infections were detected. TCR-gamma TCR-gene rearrangement was observed in 7 cases; all of them extranodal. Three of TCR-gamma(+) cases showed EBV subtype A. Two TCR-gamma(+)/CD56(+) cases showed EBV subtype B. Geographic distribution of NK/TCL showed higher frequency in the southeast and northeast regions of Brazil. Striking differences among geographic regions were seen with the vast majority of EBV subtype B (86%) occurring in the south and southeast regions.