肝脾γδT细胞淋巴瘤2例并文献复习

Hepatosplenic γδ T-cell Lymphoma:Report on A Series of 2 Patients and Correlative Literature Review

目的:探讨肝脾γδT细胞淋巴瘤的临床表现、病理学特征、免疫表型特点。方法:对我院2例确诊的肝脾γδT细胞淋巴瘤患者的临床资料进行分析、追踪随访并进行文献复习。结果:该组患者均为青年男性,肝脾不同程度长大,发热,全血细胞减少(1/2),肝功能受损,淋巴结未受累;病理示瘤细胞弥漫性肝、骨髓的窦内侵犯;免疫表型:患者瘤细胞表达CD2(+)、CD3(+)、CD56(+)、CD16(+)、CD20(-)、TIA1(+)、TCR-γ/δ(+)。结论:肝脾γδT细胞淋巴瘤是较为罕见的外周T细胞淋巴瘤,以肝脾长大、发热为主要临床表现,通常淋巴结不受累,病情进展快,疗效差,生存期短。

Objective：To investigate the clinicopathologic characteristics, immunophenotype of hepatosplenic γδT- cell lymphoma. Methods： Two patients with hepatosplenic γδ T-cell lymphoma were studied and followed up. Meanwhile we review some correlative literature. Results：The disease had uniform clinic characteristics：predominant occurrence in young male adults, hepatosplenomegaly presentation without lymphadenopathy, with fever, cytopenia（ 1/2）, hypohepatia ; Histopathologic findings were homogeneous and showed the presence of medium sized lymphoma cells within the sinusoids of liver, and hone marrow;Immunohistoch-emiealiy;lymphoma ceils were positive for CD2 ,CD3 ,CD56, CD16. Conclusion： Hepatospe nicγδ T-cell lymphoma is a rare clinicopathologic entity of peripheral T cell lymphomas. Most of patients are admitted for hepatosplenomegaly and fever without lymphadenopathy. Current treatment modalities appear to be Ineffective in most patients, prognosis poor.