Kim Min Chong, Shin Dong Hoon, Lee Jae Min
Department of Pathology, Yeungnam University Medical Center, Daegu 42415, Republic of Korea.
Department of Dermatology, College of Medicine, Yeungnam University, Daegu 42415, Republic of Korea.
Children (Basel). 2025 Aug 5;12(8):1029. doi: 10.3390/children12081029.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare subtype of cutaneous T-cell lymphoma. It is characterized by the neoplastic infiltration of subcutaneous adipose tissue. Its clinical presentation, including subcutaneous nodules, fever, and systemic symptoms, often mimics inflammatory panniculitis, making diagnosis difficult. This case report describes a 14-year-old female presenting with fever, limb pain, swelling, and subcutaneous nodules, who was ultimately diagnosed with SPTCL via punch biopsy and BIOMED-2 clonality assays, confirming positive T-cell receptor-γ chain gene rearrangement. Positron emission tomography-computed tomography revealed diffuse subcutaneous involvement across multiple body regions. Methylprednisolone and cyclosporine A treatment rapidly resolved her symptoms, with laboratory parameters, including ferritin and inflammatory markers, showing significant improvement. Next-generation sequencing identified a heterozygous C9 gene mutation (c.346C>T, p.Arg116Ter), adding a novel genetic dimension to the case. Following a tapered discontinuation of immunosuppressive therapy, the patient achieved sustained remission without relapse for over 1 year. We report a case of adolescent SPTCL treated with immunosuppressive therapy and suggest that immunosuppressive therapy should be considered before chemotherapy in pediatric patients with SPTCL but without HLH.
皮下脂膜炎样T细胞淋巴瘤（SPTCL）是皮肤T细胞淋巴瘤中一种非常罕见的亚型。其特征为皮下脂肪组织的肿瘤性浸润。其临床表现，包括皮下结节、发热和全身症状，常类似炎性脂膜炎，导致诊断困难。本病例报告描述了一名14岁女性，出现发热、肢体疼痛、肿胀和皮下结节，最终通过打孔活检和BIOMED-2克隆性分析确诊为SPTCL，证实T细胞受体γ链基因重排阳性。正电子发射断层扫描-计算机断层扫描显示多个身体区域弥漫性皮下受累。甲泼尼龙和环孢素A治疗迅速缓解了她的症状，包括铁蛋白和炎症标志物在内的实验室参数显示有显著改善。下一代测序鉴定出一个杂合的C9基因突变（c.346C>T，p.Arg116Ter），为该病例增添了新的遗传维度。在逐渐停用免疫抑制治疗后，患者实现了持续缓解，超过1年未复发。我们报告了一例接受免疫抑制治疗的青少年SPTCL病例，并建议对于患有SPTCL但无噬血细胞性淋巴组织细胞增生症（HLH）的儿科患者在化疗前应考虑免疫抑制治疗。
