肝脾T细胞淋巴瘤诊治进展

Progress in the diagnosis and treatment of hepatosplenic T-cell lymphoma

肝脾T细胞淋巴瘤是一种特殊类型的外周T细胞淋巴瘤,因其肝脾增大的病理特点及表达γδT细胞受体的分子学特征而命名。其特点是肿瘤细胞主要分布于结外,尤其是肝、脾及骨髓的窦内浸润,从而导致肝脾肿大、外周血细胞减少等一系列临床表现。该病临床罕见,高度侵袭性,临床表现无特异性,诊断及治疗十分困难,常规化疗效果差,预后极差。为提高对该疾病的认识,及早识别诊断,探讨其治疗策略,本文从病因、临床表现、诊断及治疗等方面作一综述。

Hepatosplenic T-cell lymphoma is a rare and particular peripheral T-cell lymphoma. It is named after its characteristic pathologic and molecular features, which are hepatosplenomegaly and the expression of γδT-cell receptors. As we all know, this disease is characterized by primary extranodal distribution of the malignant cells with typical intrasinusoidal infiltration of the liver, spleen, and bone marrow, which results in a series of clinical manifestations that hepatosplenomegaly and peripheral blood cytopenia and so on. It is an extremely rare, highly aggressive, non-specific disease which is difficult for diagnosis and treatment and extremely poor response to currently known conventional chemotherapy regimens, with dismal prognosis. To improve the awareness of disease and identify this disease from a variety of similar diseases, at the same time, aim at discussing treatment strategies, this article reviewed this disease from the angle of pathogeny, clinical manifestation, diagnosis and treatment.