Weidmann E
Department of Internal Medicine III, Hematology/Oncology, JW Goethe University, Frankfurt am Main, Germany.
Leukemia. 2000 Jun;14(6):991-7. doi: 10.1038/sj.leu.2401784.
Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. A rare entity within this group is represented by hepatosplenic gammadelta T cell lymphoma, which is characterized by primary extranodal disease with typical sinusoidal or sinusal infiltration of the liver and the spleen, respectively, by expression of the T cell receptor gammadelta chain, and by a number of other frequent clinicopathological features including aggressive course of disease. In contrast to these common attributes some biologic characteristics such as expression of cytotoxic proteins and cytotoxic activity have been controversial. In this review, clinicopathological, immunophenotypical, molecular biological, cytogenetical and biological findings, and diagnostic and therapeutic difficulties in hepatosplenic gammadelta T cell lymphoma are discussed.
外周T细胞淋巴瘤是一组异质性的胸腺后成熟淋巴系统恶性肿瘤，约占所有非霍奇金淋巴瘤的10%-15%。该组中的一种罕见实体是肝脾γδT细胞淋巴瘤，其特征为原发性结外疾病，肝脏和脾脏分别有典型的窦状隙或窦样浸润，表达T细胞受体γδ链，以及许多其他常见的临床病理特征，包括疾病进展迅速。与这些共同特征相反，一些生物学特性，如细胞毒性蛋白的表达和细胞毒性活性，一直存在争议。在这篇综述中，我们讨论了肝脾γδT细胞淋巴瘤的临床病理、免疫表型、分子生物学、细胞遗传学和生物学发现，以及诊断和治疗困难。
