肝脾γδT细胞淋巴瘤一例

A Case of Hepatosplenic γδ T cell lymphoma

肝脾γδT细胞淋巴瘤是一种罕见的高度恶性的外周T细胞淋巴瘤,表达T细胞标志CD2、CD3以及γδ型T细胞受体。病理表现为肿瘤性T细胞在肝脏血窦和脾脏红髓的弥漫性浸润,不形成结节,易造成误诊。通过对患者肝脏活检标本、脾脏穿刺标本和骨髓标本的免疫表型及TCR受体分析,诊断为1例成人肝脾γδT细胞淋巴瘤。结合文献复习,可以认为免疫表型及TCR受体分析是诊断肝脾γδT细胞淋巴瘤的必要手段。

Hepatosplenic γδ 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 γδ 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 γδ T cell lymphoma was diagnosed. In combination with references, It is belived that immunophenotype and TCR rearrangement are necessary means to diagnosis hepatosplenic γδ T cell lymphoma.