皮下脂膜炎样T细胞淋巴瘤10例临床分析

本研究探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床、病理特征及预后。回顾性分析了经病理证实的10例SPTCL患者的临床特点、病理形态及免疫组化标记、治疗和生存情况。结果显示:临床上首发症状均表现为单发或多发皮下结节,其中8例出现持续高热、消瘦、肝功能严重受损、累及骨髓,病程进展迅速;在病理方面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,脂肪小叶间隔无累及;肿瘤表达细胞毒T细胞的免疫表型;10例均用CHOP为主的方案化疗,总有效率60%,3年生存率为10%,全组中位生存时间10个月。结论:本病是一种以累及皮下脂肪组织为主的侵袭性淋巴瘤,且进展快,中位生存期短,伴嗜血细胞综合症者预后差,异基因造血干细胞移植为有效的治疗手段。

Clinical Analysis of 10 Patients with Subcutaneous Panniculitis-like T Cell Lymphoma

This study was purposed to evaluate the clinical and pathological features,prognosis of patients with subcutaneous pamuiculitis-time T cell lymphoma（SPTCL）.The clinicopathologic features,immunophenotypes and treatment of 10 SPTCL patients which confirmed by pathology were analyzed retrospectively.The results showed that the main clinical manifestations of SPTCL were the single or multiple subcutaneous nodules.Of them 8 cases were found with recurrent high fever,weight loss,injury of liver function,bone marrow involvement and pancytopenia.This disease rapidly advanced.Pathologically,atypical large,medium-size and small-lymphocytes rounded the lipocytes look like rosettes.The reactive proliferation of histocytes accompanied by phagothrocytic phenomena,polynuclear giant cells and granulomatoid reaction.The tumor cells infiltrated into the lipolubuls.This lymphoma expressed the cytotoxic T-cell immunophenotype.CHOP regimen was the most common chemotherapy regimen used.60% patients achieved a good initial response to chemotherapy.3-year survival was 10%,with median survival time of 10 months.It is concluded that SPTCL is a specific type of lymphoma involving primarily in subcutaneous fatty tissues,most cases of SPTCL display an aggressive course,the disease may progress rapidly and accompanies with unfavorable prognosis.And the prognosis is poor in SPTCL patients with hemophagocytic syndrome.but the allo-HSCT can improve the outcome of this disease.