九例脾脏T细胞和NK细胞淋巴瘤临床病理与免疫表型分析

Splenic T-cell and NK-cell iymphomas： a clinicopathologic and immunophenotypic analysis of 9 cases

目的探讨脾脏T细胞和NK细胞肿瘤的临床病理特征及病理诊断。方法复习9例脾脏T细胞和NK细胞肿瘤患者的临床病理资料并进行随访，进行免疫表型检测、EBER原位杂交及TCR-1基因重排检测。选用抗体有CD45RO、CD3ε、CD3、CD4、CD8、CD56、TIA-1、Granzyme B、CD30、Ki-67和CD20。结果本组9例患者中，4例为肝脾T细胞淋巴瘤，4例为结外鼻型NK／T细胞淋巴瘤，1例为非特指外周T细胞淋巴瘤。7例有随访资料，5例死亡（2例肝脾T细胞淋巴瘤，2例结外鼻型NK／T细胞淋巴瘤和1例非特指外周T细胞淋巴瘤），生存时间为1—10个月。2例存活，1例为结外鼻型NK／T细胞淋巴瘤（2月余），1例为肝脾T细胞淋巴瘤（14月余）。结论脾脏T及NK细胞肿瘤是一组少见的具有不同临床病理特征的异质性淋巴瘤，均呈侵袭性临床过程，预后差。该类肿瘤的病理诊断须结合临床、形态学、免疫表型及基因重排检测进行。

Objective To explore the clinicopathologic features and diagnosis of splenic T-cell and NK-cell neoplasms. Methods Nine cases of splenic T-cell and NK-cell neoplasms were collected and studled by morphology, immunophenotyping, EBER in situ hybridization and TCR-γ gene rearrangement. Antibodies used were as follows： CD45RO, CD3ε, CD3, CD4, CD8, CD56, TIA-1, GranzymeB, CD30, Ki-67 and CD20. Results Among the 9 cases, hepatosplenic T-cell lympboma（HSTCL） and extranodal nasal type NK/T-cell lymphoma（NK/TCL） were both of 4 cases, and the remaining one was peripheral T-cell lympboma, unspecified（PTL, unspec）. 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, unspec 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）. Conclusion Splenic T-cell and NK-cell neoplasms are a group of uncommon lympbomas with heterogeneous clinicopathologic features and poor prognosis. A definite diagnosis must depend on clinical manifestations, histopathology, immunophenotype and TCR gene rearrangement analysis.