Li Zheng, Liu Wei-Ping, Tang Yuan, Jiang Li-Li, Zhang Wen-Yan, Bi Cheng-Feng, Li Gan-Di
Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China.
Zhonghua Xue Ye Xue Za Zhi. 2007 Apr;28(4):217-22.
To explore the clinicopathologic features and diagnosis of splenic T-cell and NK-cell neoplasms.
Nine cases of splenic T-cell and NK-cell neoplasms were collected and studied by morphology, immunophenotyping, EBER in situ hybridization and TCR-gamma gene rearrangement. Antibodies used were as follows: CD45RO, CD3epsilon, CD3, CD4, CD8, CD56, TIA-1, GranzymeB, CD30, Ki-67 and CD20.
Among the 9 cases, hepatosplenic T-cell lymphoma (HSTCL) and extranodal nasal type NK/T-cell lymphoma (NK/TCL) were both of 4 cases, and the remaining one was peripheral T-cell lymphoma, unspecified (PTL, unspecified). Follow up data were available for 7 cases. Five patients including 2 with HSTCL, 2 with extranodal nasal type NK/TCL and one with PTL, unspecified died, with survival times ranged from 1 to 10 months. The other two patients are still alive, one with NK/TCL (two months+) and one with HSTCL (14+ months).
Splenic T-cell and NK-cell neoplasms are a group of uncommon lymphomas with heterogeneous clinicopathologic features and poor prognosis. A definite diagnosis must depend on clinical manifestations, histopathology, immunophenotype and TCR gene rearrangement analysis.
探讨脾脏T细胞和NK细胞肿瘤的临床病理特征及诊断方法。
收集9例脾脏T细胞和NK细胞肿瘤病例，进行形态学、免疫表型分析、EBER原位杂交及TCR-γ基因重排检测。所用抗体如下：CD45RO、CD3ε、CD3、CD4、CD8、CD56、TIA-1、颗粒酶B、CD30、Ki-67及CD20。
9例病例中，肝脾T细胞淋巴瘤（HSTCL）和结外鼻型NK/T细胞淋巴瘤（NK/TCL）各4例，其余1例为未特指的外周T细胞淋巴瘤（PTL，未特指）。7例有随访资料。5例患者死亡，包括2例HSTCL、2例结外鼻型NK/TCL和1例PTL，未特指，生存时间为1至10个月。另外2例患者仍存活，1例NK/TCL患者（2个月以上），1例HSTCL患者（14个月以上）。
脾脏T细胞和NK细胞肿瘤是一组少见的淋巴瘤，临床病理特征异质性，预后较差。明确诊断必须依靠临床表现、组织病理学、免疫表型及TCR基因重排分析。
