儿童肝脾γδT细胞淋巴瘤的临床病理分析

Clinicopathological study of hepatosplenic γδ T-cell lymphoma in childhood

目的探讨儿童肝脾γδT细胞淋巴瘤的临床病理和免疫表型特征。方法应用组织病理和免疫组化方法分析2例儿童肝脾γδT细胞淋巴瘤的骨髓和肝脏活检标本,并对相关文献进行调研。结果本文报道的2例肝脾γδT细胞淋巴瘤为9岁女孩,主要表现发热、肝脾肿大和血细胞减少,无淋巴结肿大。其中1例合并慢性活动性EB病毒感染和噬血细胞综合征,诊断时有骨髓受累。2例肝脏针刺活检病理均示中等大小非典型淋巴细胞的窦内浸润,免疫组化示CD3+CD4-CD8-Granzyme B+EBER-。结论肝脾γδT细胞淋巴瘤是一类罕见的结外侵袭性外周T细胞淋巴瘤,典型特征包括:全身症状,肝脾肿大,血细胞减少,肝脾和骨髓的窦内有CD2+CD3+CD4-CD5-CD7+CD8-TCRγδ+表型淋巴细胞的浸润,7号染色体长臂等臂畸形和8号染色体三体同时出现的细胞遗传学改变。骨髓或肝脏活检组织的免疫表型和TCR受体分析有助于此型肿瘤的诊断,目前尚无有效治疗方法。

Objective To identify the clinicopathological and immunophenotypical characteristics of hepatosplenic γδT T-cell lymphoma in childhood. Methods Two cases of hepatosplenic γδT T-cell lymphoma in childhood were studied by histopathological and inununohistochemical staining of bone marrow and liver biopsies in combination of literature review. Results We reported two 9-year-old girls with hepatosplenic γδT T-cell lymphoma. Both of them had fever, hepatosplenomegaly and cytopenia without lymphadenopathy. In one case, chronic active EBV infection and hemophagocytic syndrome were accompanied during the course of disease and bone marrow involvement was revealed at diagnosis. The histopathological examination of livers in the two cases demonstrated sinusoidal infiltration of medium-sized atypical lymphoid cells. The immunophenotype of their neoplastic cells was CD3 ^＋ CD4^- CD8^- Granzyme B ^＋ EBER^-. Conclusion Hepatosplenic γδT T-cell lymphoma is a rare, primary extranodal and aggressive entity within peripheral T-cell lymphomas, which is characterized by B symptoms, hepatosplenomegaly, cytopenia, the sinusal or sinusoidal infiltration of splenic red pulp, liver and bone marrow by neoplastic cells, the expression of the γδT T-cell receptor by tumor cells, the combination of isochromosome 7q and trisomy 8, Immunophenotyping and T-cell receptor analysis of bone marrow or liver biopsy could help in detecting this lymphoma subtype. Current treatment modalities appear to be ineffective in most patients.