Hydroa vacciniforme: a distinctive form of Epstein-Barr virus-associated T-cell lymphoproliferative disorders

Keiji Iwatsuki, Tomoko Miyake, Youji Hirai, Takenobu Yamamoto

Research output: Contribution to journalReview article

1 Citation (Scopus)

Abstract

Hydroa vacciniforme (HV) is a cutaneous subset of Epstein-Barr virus (EBV)-associated T/NK lymphoproliferative disorders (LPDs). Our previous case series study clearly showed a clinical spectrum of EBV-associated T/NK LPDs including HV, hypersensitivity to mosquito bites (HMB), chronic active EBV infection (CAEBV), and hemophagocytic lymphohistiocytosis (HLH). Patients with HV are divided into two groups: a benign subtype designated “classic HV” (cHV) and more serious systemic HV (sHV), also called “HV-like LPD” in the 2017 World Health Organization (WHO) classification. Patients with cHV usually have an increased number of EBV-infected γδT cells and patients with sHV without HMB are further classified into two groups: γδT-cell- and αβT-cell-dominant types. Patients with HMB, with or without HV-like eruptions, have an increased number of EBV-infected NK cells in the blood. Patients with cHV and γδT-cell-dominant sHV show a favourable prognosis, but the other subtypes such as αβT-cell-dominant sHV and HMB have a poor prognosis with mortality rates of 11.5 and 3.51 per 100 person-years, respectively. In addition to the clinical subtypes and the dominant lymphocyte subsets, the poor prognostic indicators include onset age over nine years and expression of the reactivation marker, BZLF1 mRNA. No prognostic correlation has been reported for anti-EBV antibody titres or EBV DNA load. The clinical subtypes and their prognostic factors should be considered for therapeutic interventions.

Original languageEnglish
Pages (from-to)21-28
Number of pages8
JournalEuropean Journal of Dermatology
Volume29
Issue number1
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Hydroa Vacciniforme
Lymphoproliferative Disorders
Human Herpesvirus 4
T-Lymphocytes
Bites and Stings
Culicidae
Hypersensitivity
Hemophagocytic Lymphohistiocytosis
Epstein-Barr Virus Infections
Lymphocyte Subsets
Viral Load
Age of Onset
Natural Killer Cells
Messenger RNA
Skin

Keywords

  • BZLF1
  • Epstein-Barr virus-associated T/NK lymphoproliferative disorders
  • hydroa vacciniforme
  • mortality
  • prognosis
  • γδT cells

ASJC Scopus subject areas

  • Dermatology

Cite this

Hydroa vacciniforme : a distinctive form of Epstein-Barr virus-associated T-cell lymphoproliferative disorders. / Iwatsuki, Keiji; Miyake, Tomoko; Hirai, Youji; Yamamoto, Takenobu.

In: European Journal of Dermatology, Vol. 29, No. 1, 01.01.2019, p. 21-28.

Research output: Contribution to journalReview article

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abstract = "Hydroa vacciniforme (HV) is a cutaneous subset of Epstein-Barr virus (EBV)-associated T/NK lymphoproliferative disorders (LPDs). Our previous case series study clearly showed a clinical spectrum of EBV-associated T/NK LPDs including HV, hypersensitivity to mosquito bites (HMB), chronic active EBV infection (CAEBV), and hemophagocytic lymphohistiocytosis (HLH). Patients with HV are divided into two groups: a benign subtype designated “classic HV” (cHV) and more serious systemic HV (sHV), also called “HV-like LPD” in the 2017 World Health Organization (WHO) classification. Patients with cHV usually have an increased number of EBV-infected γδT cells and patients with sHV without HMB are further classified into two groups: γδT-cell- and αβT-cell-dominant types. Patients with HMB, with or without HV-like eruptions, have an increased number of EBV-infected NK cells in the blood. Patients with cHV and γδT-cell-dominant sHV show a favourable prognosis, but the other subtypes such as αβT-cell-dominant sHV and HMB have a poor prognosis with mortality rates of 11.5 and 3.51 per 100 person-years, respectively. In addition to the clinical subtypes and the dominant lymphocyte subsets, the poor prognostic indicators include onset age over nine years and expression of the reactivation marker, BZLF1 mRNA. No prognostic correlation has been reported for anti-EBV antibody titres or EBV DNA load. The clinical subtypes and their prognostic factors should be considered for therapeutic interventions.",
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