皮下脂膜炎样T细胞淋巴瘤的临床特征及预后分析

Clinical Features and Prognosis of Subcutaneous Panniculitis-like T-cell Lymphoma

目的探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床表现、病理特点、治疗方案及预后。方法回顾性分析经病理证实的5例SPTCL患者的临床病理特征、诊断治疗及预后。结果 5例患者病理都存在皮下脂肪组织内中等偏大的非典型性淋巴细胞浸润,肿瘤细胞表达细胞毒T细胞的免疫表型。5例患者均有无痛性皮下结节的临床表现,2例伴发热,1例侵犯骨髓。5例患者均为晚期(Ⅲ~Ⅳ期);4例患者一般状况好(ECOG仅为1分);2例LDH升高,3例β2-MG升高。3例初治方案为CHOP,1例为BACOP,1例为BEACOP,随访至今1例死亡,1例失访,其余3例至今完全缓解。中位生存时间44(3~100)月,中位首次疾病无进展生存期8(0~96)月。结论 SPTCL发病率极低,主要累及皮下脂肪组织,目前尚无统一治疗方案,晚期患者CHOP方案疗效欠佳,含依托泊苷的高强度化疗方案可能成为有效的治疗手段。

Objective To explore the clinical manifestation, pathological features, effective treatment regimen and prognosis of subcutaneous panniculitis-like T cell lymphoma （SPTCL） patients. Methods We analyzed retrospectively the clinicopathologic features, diagnosis and treatment of five SPTCL patients who were confirmed pathologically. Results Atypical lymphocytic infiltration in the subcutaneous adipose tissue was found in all five patients. Tumor cells showed immunophenotype of cytotoxic T lymphocytes. Painless subcutaneous nodules were found in all patients： two patients with fever and one patient with bone marrow infiltration. All patients were in advanced stage（stage Ⅲ-Ⅳ）, and four patients were in good condition （ECOG score was 1）, two patients with high level of LDH and three patients with high level of β2-MG. Three cases used CHOP for initial treatment, one case used BACOP and one case used BEACOP. Up to now, one case died, one case was lost to visit, and other three cases had complete remission. The median survival time was 44 （3-100） months, and the median progression-free survival time was 8 （0-96） months. Conclusion The incidence of SPTCL is extremely low, mainly involving the subcutaneous adipose tissue. There is no uniform treatment so far. CHOP on advanced patients has poor efficacy. High intensity chemotherapy with etoposide may become the effective treatment.