Subcutaneous panniculitis‐like T‐cell lymphoma in a 14‐year‐old female homozygous for HAVCR2 mutation. (16th August 2021)

• Record Type: Journal Article
• Authors: Frederiks, Aaron J; Spagnolo, Dominic V; Ramachandran, Shanti; Brand, Roland
• Is Part Of: Australasian journal of dermatology. Volume 62:Number 4(2021)
• Page Start: e576
• Page End: e579
• Publication Date: 2021-08-16
• Subjects: subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) -- hepatitis A virus cellular receptor 2 (HAVCR2) -- T‐cell immunoglobulin mucin 3 (TIM‐3) -- p.Tyr82Cys -- p.IIe97Met -- haemophagocytic syndrome (HPS); Dermatology -- Periodicals; Dermatology -- Australasia -- Periodicals; 616.5005
• DOI: 10.1111/ajd.13684
• Languages: English
• ISSNs: 0004-8380

Abstract

ABSTRACT: 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.