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目的探讨原发性肝脾T细胞淋巴瘤的临床表现、病理学特征和免疫表型特点。方法对3例原发性肝脾T细胞淋巴瘤患者的临床资料进行回顾性分析并对相关文献进行复习。结果患者肝脾明显大,伴有发热、全血细胞减少及肝功能受损;病理示瘤细胞弥漫浸润肝/脾脏红髓血窦;免疫表型:瘤细胞表达CD+2、CD+3、CD-4、CD-8,TCR-γ/δ(+)。结论肝脾T细胞淋巴瘤是较为罕见的外周T细胞淋巴瘤,预后较差,应注意与恶性组织细胞病鉴别。
Objective To investigate the clinical manifestations, pathological features and immunophenotypic features of primary hepatosplenic T-cell lymphoma. Methods The clinical data of 3 patients with primary hepatosplenic T-cell lymphoma were analyzed retrospectively and the related literatures were reviewed. Results The liver and spleen of patients were significantly larger with fever, pancytopenia and impaired liver function. The pathology showed that the tumor cells diffusely infiltrate the liver and spleen of red blood sinusoids. Immunophenotype: The tumor cells expressed CD + 2, CD + 3, CD -4, CD-8, TCR-γ / δ (+). Conclusion Liver and spleen T-cell lymphoma is a relatively rare peripheral T cell lymphoma with poor prognosis. It should be noted that it should be differentiated from malignant histiocytosis.