腹壁皮下脂膜炎样T细胞淋巴瘤1例报道并文献复习

    Subcutaneous panniculitis-like T-cell lymphoma:one case report and review of the literature

    • 摘要: 目的:探讨皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)的临床病理特征、诊断与鉴别诊断。方法:对1例SPTCL行HE染色和免疫组织化学标记,抗体为CD3、CD45RO、CD20、CD79a、CD4、CD8、CD30、CD56、CD68、TIA-1等。结果:患者表现为发热、左中腹至左腹股沟区红肿,辅助检查提示贫血、白细胞和血小板减少、肝功能异常、肝脾肿大。镜检:肿瘤位于皮下组织,呈脂膜炎样生长,瘤细胞围绕单个脂肪细胞呈花环状排列,瘤细胞小至中等大,偶见散在的大细胞,核碎裂和核分裂象易见,并可见肿瘤性坏死、出血和脂肪坏死以及组织细胞反应。免疫表型:瘤细胞表达CD3、CD8、CD45RO、CD30、TIA-1,而CD4、CD20、CD79a、CD56、CD68阴性。患者从发病到死亡仅2月余。结论:SPTCL是一种罕见的原发于皮下脂肪组织的细胞毒T细胞淋巴瘤,一般预后较好,伴有嗜血细胞综合征和其他症状者预后可能较差。

       

      Abstract: Objective:To study the clinicopathologic features,diagnosis and differential diagnosis of subcutaneous panniculitis-like T-cell lymphoma(SPTCL).Methods:One case of SPTCL was examined by HE stainning and immunohistochemistry study.The antibodies which be used were CD3,CD45RO,CD20,CD79a,CD4,CD8,CD30,CD56,CD68 and TIA-1.Results:The case presented as fever,redness,swelling and skin ulcers in left midabdomen and the left inguinal region.Laboratory abnormalities mainly included anemia,leucopenia,thrombocytopenia,elevated liver function tests and hepatosplenomegaly.The histopathology showed a predominant subcutaneous lobular infiltrate with pleomorphic T cells of variable size with irregular and often hyperchromatic nuclei,predominantly with small to medium-sized cells with only scattered large lymphoid cells and rimming of individual adipocytes by atypical lymphocytes.Mitotic figure and karyorrhexis were present.Fat necrosis,hemorrhage,tumor-like necrosis and other cellular responses were also present in the tumor.Immunohistochemistry results showed the tumor cells were positive for CD3,CD45RO,CD8,CD30 and TIA-1,and negative for CD4,CD20,CD79a,CD56 and CD68.The patient died in two months after the onset of the disease.Conclusions:SPTCL is a rare cytotoxic T-cell lymphoma involving primarily in subcutaneous fatty tissues,it has a good prognosis commonly.But if a haemophagocytic syndrome and other syndrome is present,the prognosis maybe be poor.

       

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