Hydroa vacciniforme-like lymphoproliferative disorder in Korea

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)-associated lymphoproliferative disease. The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients....

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Veröffentlicht in:Scientific reports 2020-11, Vol.10 (1), p.19294-19294, Article 19294
Hauptverfasser: Han, Byeol, Hur, Keunyoung, Ohn, Jungyoon, Kim, Tae Min, Jeon, Yoon Kyung, Kim, You Chan, Mun, Je-Ho
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container_title Scientific reports
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creator Han, Byeol
Hur, Keunyoung
Ohn, Jungyoon
Kim, Tae Min
Jeon, Yoon Kyung
Kim, You Chan
Mun, Je-Ho
description Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein–Barr virus (EBV)-associated lymphoproliferative disease. The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients. HVLPD patients at Seoul National University Hospital between 1988 and 2019 were retrospectively analyzed. This study included 26 HVLPD patients who all presented with recurrent papulovesicular and necrotic eruption on the face, neck, and extremities. EBV was detected from the skin tissues of all patients. HVLPD was diagnosed during childhood (age
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The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients. HVLPD patients at Seoul National University Hospital between 1988 and 2019 were retrospectively analyzed. This study included 26 HVLPD patients who all presented with recurrent papulovesicular and necrotic eruption on the face, neck, and extremities. EBV was detected from the skin tissues of all patients. HVLPD was diagnosed during childhood (age &lt; 18 years) in seven patients (26.9%) and in adulthood (age ≥ 18 years) in 19 cases (73.1%). The median age at diagnosis was 24.0 years (range 7–70 years). HVLPD has various clinical courses, from an indolent course to progression to systemic lymphoma. Fourteen patients (53.8%) developed lymphoma: systemic EBV-positive T-cell lymphoma (n = 9, 34.6%); extranodal natural killer/T-cell lymphoma, nasal type (n = 3, 11.5%); aggressive natural killer/T-cell leukemia (n = 1, 3.8%); and EBV-positive Hodgkin lymphoma (n = 1, 3.8%). Mortality due to HVLPD occurred in five patients (26.3%) in the adult group, while it was one patient (14.3%) in the child group. 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The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients. HVLPD patients at Seoul National University Hospital between 1988 and 2019 were retrospectively analyzed. This study included 26 HVLPD patients who all presented with recurrent papulovesicular and necrotic eruption on the face, neck, and extremities. EBV was detected from the skin tissues of all patients. HVLPD was diagnosed during childhood (age &lt; 18 years) in seven patients (26.9%) and in adulthood (age ≥ 18 years) in 19 cases (73.1%). The median age at diagnosis was 24.0 years (range 7–70 years). HVLPD has various clinical courses, from an indolent course to progression to systemic lymphoma. 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subjects 631/67/1990
692/4028
692/699/1541
692/699/4033
692/699/67
Adolescent
Adult
Adults
Age
Aged
Child
Children
Disease Progression
Epstein-Barr virus
Epstein-Barr Virus Infections - epidemiology
Epstein-Barr Virus Infections - virology
Extremities
Female
Herpesvirus 4, Human
Hodgkin's lymphoma
Humanities and Social Sciences
Humans
Hydroa Vacciniforme - diagnosis
Hydroa Vacciniforme - epidemiology
Hydroa Vacciniforme - pathology
Hydroa Vacciniforme - virology
Immunoproliferative diseases
Leukemia
Lymphatic diseases
Lymphocytes
Lymphocytes T
Lymphoma
Lymphoma, T-Cell - epidemiology
Lymphoma, T-Cell - virology
Lymphoproliferative Disorders - epidemiology
Lymphoproliferative Disorders - virology
Male
Middle Aged
Mortality
multidisciplinary
Natural killer cells
Necrosis
Patients
Republic of Korea - epidemiology
Retrospective Studies
Science
Science (multidisciplinary)
Skin - metabolism
Skin - pathology
Skin Neoplasms - epidemiology
Skin Neoplasms - virology
T-cell lymphoma
Young Adult
title Hydroa vacciniforme-like lymphoproliferative disorder in Korea
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