皮下脂膜炎样T细胞淋巴瘤20例临床病理分析

A clinical pathological analysis of subcutaneous panniculitis-like T cell lymphoma

目的:研究皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床病理特征、免疫标记表达和预后的相关因素。方法:回顾性分析20例SPTL患者的临床病理资料,并对其存档蜡块重新进行切片,行常规组织病理学检查和免疫组化标记。结果:20例患者中男9例、女11例,中位年龄29.5岁,主要体征为无症状的结节、斑块或肿块。首次发现SPTL伴眼球突出2例。瘤细胞在皮下脂肪内呈脂膜炎样浸润,细胞大小不等、异形性明显。瘤内可见豆袋细胞、肉芽肿、小片坏死和肿瘤细胞浸润血管。瘤细胞表达T细胞标志T细胞内抗原-1(TIA-1)、颗粒酶B(GrB)、CD2、CD3、CD8、CD45RO,不表达CD4、CD20、CD56和CD68。2例患者部分大瘤细胞表达CD30。接受随访的11例患者7例死于本病。结论:SPTL有特殊的临床病理表现和免疫标记;皮损溃疡形成、并发噬血细胞综合征可能提示预后不良。

Objective： To investigate the clinical pathological features, the expression of immunohistochemical markers and the prognosis of subcutaneous panniculitis-like T cell lymphoma （SPTL）. Methods： Clinical pathological analysis and immunohistochemical staining were employed. Results： Twenty cases of SPTL were studied, which including 9 males and 11 females with median age of 29.5 years old. The main clinical manifestations were symptomless nodules, plagues and masses. The exophthalmoses were detected in two cases. Histologically, the tumor infiltration pattern was panniculitis-like in the adipose layer and the tumor cells were in different size with predominant atypia. There were beanbag cells, granulomas, pieces of necrosis and blood vessels infiltrated by tumor cells. The tumor cells were positive to CD2, CD3, CD8, CD45RO, TIA-1 and granzyme B, while were negative to CD4, CD20, CD56 and CD68. CD30 expression was found in some big tumor cells in two cases. In the 11 follow-up cases, seven of them were died of SPTL. Conclusions： SPTL has the specific clinical pathological manifestations and the tumor cells express some specific immunohistochemical markers. The ulceration on the lesion and hemophagocytic syndrome may signify the poor prognosis.