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Hydroa vacciniforme and hydroa vacciniforme‐like T‐cell lymphoma: an uncommon event for transformation
Author(s) -
Chen ChienChin,
Chang KungChao,
Medeiros L. Jeffrey,
Lee Julia YuYun
Publication year - 2016
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12801
Subject(s) - lymphoma , epstein–barr virus , medicine , pathology , scars , t cell lymphoma , hemophagocytosis , virus , immunohistochemistry , monoclonal , cutaneous lymphoma , epstein–barr virus infection , dermatology , immunology , antibody , mycosis fungoides , monoclonal antibody , bone marrow , pancytopenia
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.