Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma: an uncommon event for transformation

doi:10.1111/cup.12801

CNU IR > Pharmacy and Science > Dept. of Cosmetic Science and institute of cosmetic science > Periodical Articles > Item 310902800/30997

Please use this identifier to cite or link to this item: https://ir.cnu.edu.tw/handle/310902800/30997

Title:	Hydroa vacciniforme and hydroa vacciniforme-like T-cell lymphoma: an uncommon event for transformation
Authors:	Chen, Chien-Chin Chang, Kung-Chao Medeiros, L. Jeffrey Lee, Julia Yu-Yun
Contributors:	Chia Yi Christian Hosp, Dept Pathol Chia Nan Univ Pharm & Sci, Dept Cosmet Sci Natl Cheng Kung Univ & Hosp, Dept Pathol, Coll Med Univ Texas MD Anderson Canc Ctr, Dept Hematopathol Natl Cheng Kung Univ & Hosp, Dept Dermatol, Coll Med
Keywords:	Epstein-Barr virus HV-like T-cell lymphoma hydroa vacciniforme (HV) T-cell clonality transformation pathway
Date:	2016-12
Issue Date:	2018-01-18 11:39:30 (UTC+8)
Publisher:	Wiley-Blackwell
Abstract:	Background: Hydroa vacciniforme (HV) is associated with Epstein-Barr virus (EBV) infection and a risk of transformation to lymphoma. Methods: We retrospectively analyzed six HV cases for EBV association and transformation to HV-like T-cell lymphoma. Clinicopathologic features were reviewed and cases were assessed for EBV-encoded RNA (EBER) by in situ hybridization, double staining with immunohistochemistry and EBER and for T-cell clonality. Results: The male-to-female ratio was 5: 1, with a median age at diagnosis of 18.5 years. All patients initially had recurrent vesicles, necrotic ulcers or scars on sun-exposed areas. Symptoms were present before diagnosis between 2 weeks to 10 years. The mean follow-up time was 106.3 months. Four patients (67%) were EBV-positive. All four EBV-positive and one EBV-negative patients had relapsing clinical course. Double staining proved EBV infection in T-cells. Moreover, one EBV-positive patient developed HV-like T-cell lymphoma with hemophagocytosis after 209 months of recurrent papulovesicular eruptions and eventually died. T-cell clonality was successfully performed in four HV patients and all showed polyclonal results; the transformed HV-like T-cell lymphoma was monoclonal. Conclusions: In EBV endemic areas, HV is frequently (67%) associated with EBV infection, but transformation to HV-like T-cell lymphoma seems to be uncommon (17%) and bear a dismal outcome.
Relation:	Journal of Cutaneous Pathology, v.43 n.12, pp.1102-1111
Appears in Collections:	[Dept. of Cosmetic Science and institute of cosmetic science] Periodical Articles

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