Louisiana State University, New Orleans, LA, USA.
J Investig Med High Impact Case Rep. 2020 Jan-Dec;8:2324709620981531. doi: 10.1177/2324709620981531.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare peripheral cytotoxic T-cell lymphoma, clinically resembling panniculitis. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune overactivation, triggered by underlying conditions. SPTCL presenting with HLH may represent a severe and rapidly progressive disease course. Currently, there is no standardized approach to treatment of HLH secondary to underlying SPTCL. A 34-year-old Asian male presented with a several months history of high fevers, weight loss, and nonpruritic purple discoloration of the skin. He had a skin biopsy showing atypical lymphohistiocytic panniculitis with dermal mucinosis and erythrophagocytosis consistent with SPTCL. The patient was initiated on treatment with dexamethasone and cyclosporine A. Almost immediate improvement of his skin lesions was noted and laboratory abnormalities trended toward baseline within 2 weeks. He noted complete symptom resolution after 3 months on therapy. SPTCL may be treated effectively with cyclosporine A and steroids to achieve rapid clinical and symptom management of this rare malignancy.
皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）是一种罕见的外周细胞毒性 T 细胞淋巴瘤，临床上类似于脂膜炎。噬血细胞性淋巴组织细胞增生症（HLH）是一种由潜在疾病引起的免疫过度激活的危及生命的综合征。表现为 HLH 的 SPTCL 可能代表一种严重且快速进展的疾病过程。目前，对于继发于 SPTCL 的 HLH 尚无标准化的治疗方法。一名 34 岁的亚裔男性因高热、体重减轻和非瘙痒性紫色皮肤变色而就诊，病史长达数月。他的皮肤活检显示非典型淋巴组织细胞性脂膜炎，伴有真皮粘蛋白沉积和红细胞吞噬作用，符合 SPTCL 的表现。患者开始接受地塞米松和环孢素 A 治疗。几乎立即注意到皮肤病变得到改善，并且在 2 周内实验室异常趋势接近基线。他在接受治疗 3 个月后完全缓解症状。SPTCL 可以通过环孢素 A 和类固醇有效治疗，以快速控制这种罕见恶性肿瘤的临床和症状。
