Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2021 Nov;62(4):e576-e579.
doi: 10.1111/ajd.13684. Epub 2021 Aug 16.

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

Aaron J Frederiks  1 , Dominic V Spagnolo  2   3 , Shanti Ramachandran  4   5 , Roland Brand  1
Affiliations
Case Reports

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

Aaron J Frederiks et al. Australas J Dermatol. 2021 Nov.

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

PubMed Disclaimer

References

REFERENCES

    1. Jaffe E, Gaulard P, Cerroni L. Subcutaneous panniculitis-like T-cell lymphoma. In: Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S, Stein H, Thiele J (eds). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised, 4th edn). Lyon: IARC, 2017; 383-5.
    1. Willemze R, Jansen PM, Cerroni L et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 2008; 111: 838.
    1. Michonneau D, Petrella T, Ortonne N et al. Subcutaneous panniculitis-like T-cell lymphoma: immunosuppressive drugs induce better response than polychemotherapy. Acta dermato-venereol. 2017; 97: 358.
    1. López-Lerma I, Peñate Y, Gallardo F et al. Subcutaneous panniculitis-like T-cell lymphoma: clinical features, therapeutic approach, and outcome in a case series of 16 patients. J. Am. Acad. Dermatol. 2018; 79: 892-8.
    1. Gayden T, Sepulveda FE, Khuong-Quang D-A et al. Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. Nat. Genet. 2018; 50: 1650.

Publication types

Substances

Supplementary concepts

LinkOut - more resources