Subcutaneous panniculitis‐like T‐cell lymphoma in a 14‐year‐old female homozygous for HAVCR2 mutation

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...

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Bibliographic Details
Published in:Australasian journal of dermatology 2021-11, Vol.62 (4), p.e576-e579
Main Authors: Frederiks, Aaron J, Spagnolo, Dominic V, Ramachandran, Shanti, Brand, Roland
Format: Article
Language:English
Subjects:
Adolescent
Cytotoxicity
Female
Genetic counseling
haemophagocytic syndrome (HPS)
hepatitis A virus cellular receptor 2 (HAVCR2)
Hepatitis A Virus Cellular Receptor 2 - genetics
Humans
Lymphoma
Lymphoma, T-Cell - genetics
Lymphoma, T-Cell - pathology
Lymphoma, T-Cell - therapy
Mutation
Mutation - genetics
p.IIe97Met
p.Tyr82Cys
Panniculitis - genetics
Panniculitis - pathology
Panniculitis - therapy
Patients
Remission
Steroid hormones
subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL)
T-cell lymphoma
T‐cell immunoglobulin mucin 3 (TIM‐3)
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Summary: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.
ISSN:0004-8380
1440-0960
DOI:10.1111/ajd.13684