Abstract
BackgroundHydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia. It can progress to systemic lymphoma. However, prognostic factors and treatment remain unclear.MethodsThis retrospective study reviewed the clinical, morphologic, immunophenotypical features, and clinical treatment of 19 patients with HV-LPD.ResultsAll 19 patients had skin lesions in the face, extremities, or areas unexposed to the sun, including edema, blistering, ulceration, and scarring. The course was slowly progressive and relapsing. Histopathology showed an atypical lymphocytic infiltrate in the dermis and/or subcutaneous tissue. The lesions had a cytotoxic T/NK-cell immunophenotype. Among 19 patients, 7 (37%) exhibited CD4+ T cells, 5 (26%) exhibited CD8+ T cells, and 7 (37%) exhibited CD56+ cells. Of 12 cases with a T-cell phenotype, molecular analyses demonstrated that 7 had monoclonal rearrangements in the T-cell receptor genes. Three cases had an NK-cell phenotype and had polyclonal rearrangements in the TCR genes. All cases were associated with EBV infections. Among 19 patients, 9 (47.4%) received chemotherapy. Only one patient received allogeneic transplantation and EBV-specific cytotoxic T lymphocyte treatment after chemotherapy. That patient was the only one alive without disease at the latest follow up. Nine patients died of systemic lymphoma with disease progression, indicating irreversible process.ConclusionsThis study confirmed that HV-LPD is a broad-spectrum EBV+ lymphoproliferative disorder. It progressed to EBV+ systemic T/NK lymphoma, although some patients had a more indolent, chronic course. Cytopenia, elevated lactate dehydrogenase, destructive-multiorgan involvement, and older age were poor prognostic factors. Only allogeneic transplantation was curative.
Highlights
Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) was nominated and introduced into the 4th revised edition of the WHO classification of Tumors of Hematopoietic and Lymphoid Tissues [1]
HV-LPD is a cutaneous form of Epstein-Barr Virus-positive (EBV+) T/NK lymphoproliferative disease, which occurs in childhood and carries the risk of systemic lymphoma [2, 3]
Most skin lesions initially occurred after sun exposure and subsided after sun protection
Summary
Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) was nominated and introduced into the 4th revised edition of the WHO classification of Tumors of Hematopoietic and Lymphoid Tissues [1]. HV-LPD is a cutaneous form of Epstein-Barr Virus-positive (EBV+) T/NK lymphoproliferative disease, which occurs in childhood and carries the risk of systemic lymphoma [2, 3]. It manifests as recurrent skin lesions infiltrated with T cells or NK cells. It is mostly found in children and young adults from Asian and Latin American countries. Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barr virus (EBV) infection, which occurs mainly in children in Latin America and Asia.
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