Chien-Chin Chen1,2, Kung-Chao Chang3, L Jeffrey Medeiros4, Julia Yu-Yun Lee5. 1. Department of Pathology, Chia-Yi Christian Hospital, Chia-Yi, Taiwan. 2. Department of Cosmetic Science, Chia Nan University of Pharmacy and Science, Tainan, Taiwan. 3. Department of Pathology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan. 4. Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. 5. Department of Dermatology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan.
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.
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.
Authors: Melissa A Levoska; Jeffrey I Cohen; Irini Manoli; Chyi-Chia Richard Lee; Steven S T Ching; Jessica Shand; Deborah Tamura; Kenneth H Kraemer; John J DiGiovanna Journal: J Am Acad Dermatol Date: 2017-09-28 Impact factor: 11.527
Authors: Elżbieta Grześk; Sylwia Kołtan; Anna Dąbrowska; Anna Urbańczyk; Jadwiga Małdyk; Bogdan Małkowski; Tomasz Bogiel; Robert Dębski; Krzysztof Czyżewski; Mariusz Wysocki; Jan Styczyński Journal: Front Immunol Date: 2022-08-05 Impact factor: 8.786