皮下脂膜炎样T细胞淋巴瘤2例报告并文献复习

目的：探讨皮下脂膜炎样T细胞淋巴瘤（SPTCL）的临床和病理特征。方法：对2例SPTCL的临床资料进行分析，并结合文献复习，为其诊断和鉴别诊断提供一条思路。结果：临床上首发症状均表现为皮下结节，单发或多发。全身表现多种多样，但均出现持续高热、消瘦、肝功能严重受损，累及骨髓，伴致死性嗜血细胞综合征，病程进展迅速，生存不超过1年；病理检查见皮下脂肪组织内有原发的小、中或大的多形性T细胞，围绕脂肪细胞呈花环状外观。周围区组织细胞反应性增生活跃，伴有吞噬红细胞现象，并见多核巨细胞和肉芽肿样反应。瘤细胞浸润在脂肪小叶内，而脂肪小叶间隙无累及。肿瘤表达细胞毒T细胞的免疫表现。结论：SPTCL是一种以累及皮下脂肪组织为主的特殊类型的淋巴瘤，伴嗜血细胞综合征者预后差。

Subcutaneouspanniculitis-like T cell lymphoma:a report of 2 cases and literature review

Objective:To inquire into the clinical and pathological features of SPTCL.Method:Two cases of SPTCL and analyzed their histopathological and immunohistochemical features,were rerorted and reviewed literatures in order to give some advice for diagnosis.Result:The main clinical manifestations of SPTCL was the single or multiple subcutaneous nodules.Systemic symptoms were variable,both of cases got recurrent high fever,weight loss,and had injury to liver function.Patients with bone marrow involvement may have a lethal hemophagocytic syndrome with pancytopenia.They rapidly advanced and died less than 1 year.Pathologially,atypical large,midium-size and small lymphocytes with variable ratio rounded the lipocytes like rosettes.The histocytes reactively proliferated accompanied with phagorthrocytic phenomena,and polynuclear giant cells and granulomatoid reaction were found.The tumor cells infiltrated the lipolubuls.They expressed the cytotoxic T-cell immunophenotype.Conclusion:SPTCL is a specific type of lymphoma involving primarily in subcutaneous fatty tissues, and the prognosis is poor in those with hemophagocytic syndrome.