皮下脂膜炎样T细胞淋巴瘤2例

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 例具有突出消化系统表现的SPTCL临床资料进行分析，并结合文献复习，为其诊断和治疗提供思路。结果:SPTCL临床上首发症状多表现为皮下结节，全身表现有持续高热、凝血功能障碍、浆膜腔积液等，消化系统表现可表现为肝功能损害、肝脾肿大、大量腹水、便血等；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，有脂膜炎样改变，围绕脂肪细胞呈花环状外观。肿瘤表达T 细胞相关免疫表型，CD56多为阴性。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，确诊赖于皮下结节活检和免疫组化检查；皮下结节有特殊影像学改变，应用超声引导下对皮下结节活检可能获得良好效果；治疗方案可选择化疗、放疗或联合放化疗，合并噬血细胞综合征（HPS）者联合自身干细胞移植可能疗效更佳。

Two cases of subcutaneous panniculitis-like T cell lymphoma with a review of literature

Objective :To investigate the clinical and pathological features of SPTCL. Methods: Two cases of SPTCL representing digestive manifestation and analyzed their histopathological and immunohistochemical features were reported and reviewed literatures in order to give some advice for diagnosis and treatment. Result: The first main clinical manifestation of SPTCL was the single or multiple subcutaneous nodules. Systemic symptoms were variable, including of cases got recurrent high fever ,weight loss , and dropsy of serous cavity . Manifestations of digestive system were observed, such as liver function injury, hepatosplenomegaly, ascites, hematochezia. Pathologially, atypical large ,medium size and small lymphocytes with variable ratio rounded the lipocytes like rosettes. The tumor cells infiltrated the lipolubuls and expressed the cytotoxic T-cell immunophenotype. CD56- negative tumors were more common. Conclusion:SPTCL is a specific type of lymphoma involving primarily in subcutaneous fatty tissues, subcutaneous nodes biopsy and immunohistochemisty was necessary for diagnosis. Special finding would be observed with ultrasound (US) and computed tomography (CT) scanning in subcutaneous nodes. There were many advantages of ultrasound-guided subcutaneous nodes biopsy method. Radiotherapy , chemotherapy ,or combination radiotherapy and chemotherapy should be treatment options for SPTCL patients. Combination chemotherapy and Stem Cell Transplantation for patients with hemophagocytic syndrome(HPS) would be better.