Department of Dermatology, Perth Children's Hospital, Nedlands, Australia.
PathWest Laboratory Medicine, Nedlands, Australia.
Australas J Dermatol. 2021 Nov;62(4):e576-e579. doi: 10.1111/ajd.13684. Epub 2021 Aug 16.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma preferentially involving subcutis. A link between patients with SPTCL and HAVCR2 mutations has recently been discovered. We present a 14-year-old girl of Chinese heritage who was diagnosed with SPTCL in the context of homozygous HAVCR2 status for c.245A>G p. (Tyr82Cys) and achieved complete remission after treatment with cyclosporin and steroids. Dermatologists should be aware of the diagnostic, management and familial genetic counselling utility of HAVCR2 for investigating and managing patients with SPTCL.
皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）是一种罕见的细胞毒性 T 细胞淋巴瘤，主要累及皮下组织。最近发现 SPTCL 患者与 HAVCR2 突变之间存在关联。我们报告了一名 14 岁的华裔女孩，她在 HAVCR2 状态纯合子（c.245A>G p. （Tyr82Cys））的情况下被诊断为 SPTCL，并在接受环孢素和类固醇治疗后达到完全缓解。皮肤科医生应该意识到 HAVCR2 在诊断、管理和家族遗传咨询方面的效用，以调查和管理 SPTCL 患者。
