皮下脂膜炎样T细胞淋巴瘤临床分析

Clinicopathological analysis of subcutaneous panniculitis-like T-cell lymphoma

目的探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床病理特点及治疗策略。方法回顾性分析10例SPTCL患者的临床病理及治疗随访资料。结果SPTCL以皮下多发结节或斑块为主要临床症状,多见于下肢。中位年龄35.5岁,5例出现B型症状,6例淋巴结肿大,2例骨髓受累。病理结果显示,皮下脂肪组织浸润的淋巴细胞为非典型淋巴细胞,如小叶性或弥漫性脂膜炎,典型的免疫表型为CD3+、CD4-、CD8+和βF1+。荧光原位杂交显示EBER阴性,7例行TCR基因重排检测,其中6例阳性。10例SPTCL患者均接受了联合化疗,总有效率为100%。3例出现复发,其中1例接受挽救化疗,2例合并噬血细胞综合征(HLH),给予对症治疗。截至2021年11月,8例患者存活,2例患者死于HLH。结论SPTCL预后良好,伴有HLH的患者呈侵袭性病程。对于初治或复发的SPTCL患者来说,GDP或DDGP方案可能是一个合适的选择。

Objective To investigate the clinicopathological features and treatment of subcutaneous panniculitis-like T-cell lymphoma(SPTCL).Methods The clinicopathological,therapeutic and follow-up data of 10 patients with SPTCL confirmed by pathology were analyzed retrospectively.Results The main clinical symptoms of SPTCL are multiple subcutaneous nodules or plaques,which are common in the lower extremities.At the time of diagnosis,the median age was 35.5 years old,5 patients had B symptoms,6 patients had enlarged lymph nodes,and 2 patients had bone marrow involvement.The pathological findings showed that atypical lymphocytes infiltrated subcutaneous adipose tissue,such as lobular or diffuse panniculitis,and the typical immunophenotype was CD3+,CD4-,CD8+,andβF1+.Fluorescence in situ hybridization showed EBER negativity,and 7 patients were detected by TCR gene rearrangement,of which 6 patients were positive.All 10 patients with SPTCL received combined chemotherapy,with a total efficacy rate of 100%.3 patients experienced relapse,of which 1patient received salvage chemotherapy and 2 patients complicated with hemophagocytic lymphohistiocytosis(HLH)received symptomatic treatment.As of November 2021,8 patients survived,and 2 patients died of HLH.Conclusion The prognosis of SPTCL is good,and patients with HLH have an aggressive disease course.For newly diagnosed or relapsed SPTCL patients,GDP or DDGP regimen may be an appropriate choice.