肝脾T细胞淋巴瘤诊治进展.pdf

中华临床医师杂志( 电子版)2017 年1 月第11 卷第1 期 Chin J Clinicians(Electronic Edition),January 1,2017,Vol.11,No.1 ²127 ² ²综述² 肝脾T 细胞淋巴瘤诊治进展 刘朵平 田卫伟 马梁明 赵汀梓 赵静萍 【摘要】 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。 【关键词】 脾肿瘤； 淋巴瘤，T 细胞，外周； γδT 细胞淋巴瘤 Progress in the diagnosis and treatment of hepatosplenic T-cell lymphoma Liu Duoping, Tian Weiwei, Ma Liangming, Zhao Tingzi, Zhao Jingping. Department of Haematology, the Affiliated Dayi Hospital of Shanxi Medical University, Taiyuan 030032, China Corresponding author: Ma Liangming, Email: maliangming620928@163.com 【Abstract 】 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, diagnosi