Zhang Shuang, Nong Lin, Ren Ya-li, Li Ting
Department of Pathology, Peking University First Hospital, Beijing 100034, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2008 Aug 18;40(4):387-91.
To explore the clinicopathologic features and diagnostic criteria of hepatosplenic T-cell lymphoma (HSTCL).
Three cases of HSTCL were searched for morphology, immunophenotypings, Epstein-Barr virus (EBV) in situ hybridization and T-cell receptorgamma (TCRgamma) gene rearrangement.
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.
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.
探讨肝脾T细胞淋巴瘤（HSTCL）的临床病理特征及诊断标准。
对3例HSTCL进行形态学、免疫表型分析、爱泼斯坦-巴尔病毒（EBV）原位杂交及T细胞受体γ（TCRγ）基因重排检测。
骨髓中，1例肿瘤细胞浸润方式为间质和窦状隙浸润，另2例为弥漫性浸润。1例肝脏和1例脾脏中，肿瘤细胞分别呈窦状隙浸润。免疫表型分析：3例CD3和TIA-1均呈强阳性，但颗粒酶B、CD56和TCRβ均为阴性。所有病例EBV原位杂交均未检测到。2例检测到TCRγ单克隆重排。
HSTCL是一种罕见的外周T细胞淋巴瘤，被视为未活化细胞毒性T细胞淋巴瘤的一个亚型。EBV原位杂交阴性及TCRγ基因单克隆重排有助于HSTCL的诊断及鉴别诊断。这3例与国际报道病例表现相似。
