Background

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma of mature cytotoxic T cells. Initially, patients with SPTCL were treated with doxorubicin-based polychemotherapy.

Objective

To analyze clinical, biologic, immunophenotypical, molecular, imaging, treatment, and outcome data reflecting the current state of knowledge.

Methods

A retrospective multicenter study of 16 patients with SPTCL that was diagnosed between 1996 and 2016.

Results

The female-to-male ratio was 1.7. The median age at diagnosis was 46.5 years. Patients presented with multiple nodular or plaque-like lesions preferentially affecting the legs and/or trunk. Histopathology typically showed a lobular panniculitis with individual adipocytes surrounded by atypical lymphocytes, usually with a CD3⁺, CD4^–, CD8⁺, CD56^–, TIA1 cytotoxic granule associated RNA binding protein 1–positive phenotype and high proliferation rate. SPTCL was associated with autoimmune diseases in 25% of patients, and with the development of hemophagocytic syndrome in 18% of patients. Oral steroids alone or in combination with low-dose methotrexate or cyclosporine A were the most common initial treatment, achieving a complete response in 85% of the treated patients. The median follow-up time was 14 months. The 5-year disease-specific survival rate was 85.7%.

Limitations

This was a retrospective study.

Conclusions

SPTCL has an excellent prognosis. Immunosuppressive agents can be considered for first-line treatment.

中文翻译：

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皮下脂膜炎样T细胞淋巴瘤：16例患者的临床特征，治疗方法和预后

背景

皮下脂膜炎样T细胞淋巴瘤（SPTCL）是成熟的细胞毒性T细胞罕见的原发性皮肤淋巴瘤。最初，患有SPTCL的患者接受了以阿霉素为基础的多化学疗法治疗。

客观的

分析反映当前知识状态的临床，生物学，免疫表型，分子，影像学，治疗和结果数据。

方法

在1996年至2016年之间对16例SPTCL患者进行了一项回顾性多中心研究。

结果

男女比例为1.7。诊断时的中位年龄为46.5岁。出现多个结节或斑块样病变的患者会优先影响腿部和/或躯干。组织病理学通常呈小叶脂膜炎与个别脂肪细胞包围异型淋巴细胞，一般用CD3 ⁺，CD4 ^-，CD8 ⁺，CD56 ^-，TIA1细胞毒性颗粒相关的RNA结合蛋白1阳性表型和高增殖率。SPTCL在25％的患者中与自身免疫性疾病相关，在18％的患者中与噬血细胞综合征相关。单独使用口服类固醇或与小剂量甲氨蝶呤或环孢素A联合使用是最常见的初始治疗，在85％的治疗患者中可实现完全缓解。中位随访时间为14个月。5年疾病特异性存活率为85.7％。

局限性

这是一项回顾性研究。

结论

SPTCL预后良好。免疫抑制剂可考虑用于一线治疗。