Gaulard P, Zafrani E S, Mavier P, Rocha F D, Farcet J P, Divine M, Haioun C, Pinaudeau Y
Hepatology. 1986 Sep-Oct;6(5):864-8. doi: 10.1002/hep.1840060510.
Three cases of a peculiar form of peripheral T-cell lymphoma presenting as predominant hepatic disease with splenomegaly are reported. The three patients had marked liver enlargement without lymphadenopathy; white blood cell count was normal, and modifications of hepatic tests were mild. In the three cases, the diagnosis of the lymphoma was mainly based on the results of hepatic morphological changes. Liver involvement was histologically characterized by a predominantly sinusoidal infiltration by tumor cells in the three cases, associated with perisinusoidal fibrosis in two of them; portal infiltration was noted in two patients. Immunopathological study showed that tumor cells were T-lymphoid cells that were different from normal T-lymphocytes by the lack of expression of one T-cell membrane antigen, i.e., Leu-1. These findings suggest that a distinct clinical, pathological and immunopathological entity might be individualized within the large group of T-cell lymphomas.
报告了3例表现为以肝脏疾病为主并伴有脾肿大的特殊类型外周T细胞淋巴瘤。这3例患者肝脏明显肿大，无淋巴结病；白细胞计数正常，肝功能检查改变轻微。在这3例病例中，淋巴瘤的诊断主要基于肝脏形态学改变的结果。3例病例的肝脏受累在组织学上的特征均为肿瘤细胞主要呈窦状浸润，其中2例伴有窦周纤维化；2例患者可见门脉浸润。免疫病理学研究显示，肿瘤细胞为T淋巴细胞，因缺乏一种T细胞膜抗原即Leu-1的表达而与正常T淋巴细胞不同。这些发现提示，在一大组T细胞淋巴瘤中可能存在一种独特的临床、病理和免疫病理实体。
