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

Aaron J. Frederiks; Dominic V. Spagnolo; Shanti Ramachandran; Roland Brand

doi:10.1111/ajd.13684

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

Aaron J. Frederiks, Dominic V. Spagnolo, Shanti Ramachandran, Roland Brand

Pathology & Laboratory Medicine

Research output: Contribution to journal › Review article › peer-review

4 Citations (Web of Science)

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.

Original language	English
Pages (from-to)	e576 - e579
Journal	Australasian Journal of Dermatology
Volume	62
Issue number	4
DOIs	https://doi.org/10.1111/ajd.13684
Publication status	Published - Nov 2021

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title = "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 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.",

keywords = "haemophagocytic syndrome (HPS), hepatitis A virus cellular receptor 2 (HAVCR2), p.IIe97Met, p.Tyr82Cys, subcutaneous panniculitis-like T-cell lymphoma (SPTCL), T-cell immunoglobulin mucin 3 (TIM-3)",

author = "Frederiks, {Aaron J.} and Spagnolo, {Dominic V.} and Shanti Ramachandran and Roland Brand",

year = "2021",

month = nov,

doi = "10.1111/ajd.13684",

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journal = "Australasian Journal of Dermatology",

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TY - JOUR

T1 - Subcutaneous panniculitis-like T-cell lymphoma in a 14-year-old female homozygous for HAVCR2 mutation

AU - Frederiks, Aaron J.

AU - Spagnolo, Dominic V.

AU - Ramachandran, Shanti

AU - Brand, Roland

PY - 2021/11

Y1 - 2021/11

N2 - 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.

AB - 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.

KW - haemophagocytic syndrome (HPS)

KW - hepatitis A virus cellular receptor 2 (HAVCR2)

KW - p.IIe97Met

KW - p.Tyr82Cys

KW - subcutaneous panniculitis-like T-cell lymphoma (SPTCL)

KW - T-cell immunoglobulin mucin 3 (TIM-3)

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U2 - 10.1111/ajd.13684

DO - 10.1111/ajd.13684

M3 - Review article

C2 - 34398459

AN - SCOPUS:85112455146

SN - 0004-8380

VL - 62

SP - e576 - e579

JO - Australasian Journal of Dermatology

JF - Australasian Journal of Dermatology

IS - 4

ER -

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

Abstract

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