Wang Fu-Xu, Zhang Xue-Jun, Dong Zuo-Ren
Department of Hematology, The Second Hospital, Hebei Medical University, Shijiazhuang 050000, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2005 Jun;13(3):505-8.
Hepatosplenic gammadelta T cell lymphoma represents rare, often aggressive type of malignant peripheral T-cell lymphoma, which is characterized by expressing T-cell-associated markers CD2, CD3 and gammadelta T-cell receptor, and nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). The pathological findings of a liver biopsy specimen revealed the diffuse infiltration of lymphocytes in the sinusoids and the aspiration biopsy from spleen revealed the diffuse infiltration of lymphocytes in the red pulp, not shaped to the nodes, often resulted in the misdiagnosis. Recently, by analyzing the immunophenotype and TCR rearrangement from liver, spleen and bone marrow, a case of adult hepatosplenic gammadelta T cell lymphoma was diagnosed. In combination with references, It is belived that immunophenotype and TCR rearrangement are necessary means to diagnosis hepatosplenic gammadelta T cell lymphoma.
肝脾γδ T细胞淋巴瘤是一种罕见的、通常具有侵袭性的恶性外周T细胞淋巴瘤，其特征是表达T细胞相关标志物CD2、CD3和γδ T细胞受体，以及非活化的细胞毒性细胞表型（TIA-1+，颗粒酶B-）。肝活检标本的病理结果显示淋巴细胞在肝血窦中弥漫浸润，脾穿刺活检显示淋巴细胞在脾红髓中弥漫浸润，不成结节状，常导致误诊。最近，通过分析肝脏、脾脏和骨髓的免疫表型和TCR重排，诊断出一例成人肝脾γδ T细胞淋巴瘤。结合文献，认为免疫表型和TCR重排是诊断肝脾γδ T细胞淋巴瘤的必要手段。
