肝脾T细胞淋巴瘤临床病理学分析

目的:探讨肝脾T细胞淋巴瘤(hepatosplenic T cell lymphoma,HSTCL)的临床病理特征和病理诊断.方法:复习3例HSTCL患者的临床病理资料,免疫表型,EB病毒(Epstein-Barr virus,EBV)原位杂交及T细胞受体γ (T cell receptor γ,TCBγ)基因重排检测.结果:例HSTCL中,骨髓1例呈间质和窦性浸润,2例呈间质及弥漫性浸润;肝1例和脾1例呈窦性浸润.免疫组化:例均CD3、TIA-1强阳性,而粒酶B阴性;CD56阴性;TCRβ阴性;3例EBV原位杂交均阴性.TCRγ基因重排检测2例呈单克隆性,1例呈弥散态.结论:STCL是一种罕见的非活化T细胞毒性的外周T细胞淋巴瘤(peripheral T cell lymphoma,PTCL),EBV原位杂交阴性和TCRγ脚基因克隆性重排有助于诊断和鉴别诊断.我国病例与国际报道病例临床病理特征基本一致.

Clinicopathologic study of hepatosplenic T-cell lymphoma

OBJECTIVE:To explore the clinicopathologic features and diagnostic criteria of hepatosplenic T-cell lymphoma (HSTCL). METHODSú Three cases of HSTCL were seached for morphology, immunophenotypings, Epstein-Barr virus (EBV) in situ hybridization and T-cell receptorgamma (TCRgamma) gene rearrangement. RESULTSú In the bone marrow, the infiltrative pattern of tumour cells was interstitial and sinusoidal infiltration in one caseìwhile the other two cases showed diffuse infiltration. In the liver of one case and the spleen of another case, tumour cells respectively showed sinusoidal infiltration. The immunophenotyping: three cases showed strongly positive for CD3 and TIA-1, but negative for Granzyme B, CD56 and TCRbeta. EBV in situ hybridization was not detected in all the cases. TCRgamma monoclonal rearrangements were detected in two cases. CONCLUSIONú HSTCL is a rare entity which is classified into peripheral T cell lymphomas. And it is regarded as a subset of unactived cytotoxic T-cell lymphomas. The negative results of EBV in situ hybridization and the presentation of TCRgamma gene monoclonal rearrangements may be helpful in diagnosis and differential diagnosis of HSTCL. These three cases showed similar characters to those of international cases reported.