Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites

Tomoko Miyake, T. Yamamoto, Youji Hirai, M. Otsuka, T. Hamada, K. Tsuji, Shin Morizane, D. Suzuki, Y. Aoyama, K. Iwatsuki

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

Background Epstein-Barr virus (EBV)-associated T/natural-killer lymphoproliferative disorders form a group of diseases that includes classical and systemic hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). Patients with systemic HV (sHV) and HMB often have a poor prognosis, although little is known about the prognostic factors. Objectives To elucidate the prognostic factors of HV and HMB. Methods We studied clinicopathological manifestations, routine laboratory findings, anti-EBV titres, EBV DNA load and EBV-encoded gene expression, including expression of BZLF1, in 50 patients with classical HV (cHV), sHV, HMB only and HMB with HV (HMB + HV), and further analysed 30 patients who were available for follow-up. Results The median age of disease onset was 5 years (range 1-74). A follow-up study indicated that fatal outcomes were observed in three of eight patients with sHV, two of six patients with HMB only, and two of five patients with HMB + HV. The main causes of death were complications from haematopoietic stem-cell transplantation and multiorgan failure. There were no fatalities among the 11 patients with cHV. Univariate analysis revealed two poor prognostic indicators: (i) onset age > 9 years and (ii) the expression of an EBV-encoded immediate-early gene transcript, BZLF1 mRNA, in the skin lesions (P <0·001 and P = 0·003, respectively). Conclusions No prognostic correlation was observed in EBV-infected lymphocyte subsets, anti-EBV antibody titres or EBV DNA load. Late onset and EBV reactivation are both related to more severe phenotypes of the disease, and thus may predict a poor prognosis.

Original languageEnglish
Pages (from-to)56-63
Number of pages8
JournalBritish Journal of Dermatology
Volume172
Issue number1
DOIs
Publication statusPublished - Jan 1 2015

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Hydroa Vacciniforme
Bites and Stings
Culicidae
Human Herpesvirus 4
Hypersensitivity
Survival Rate
Viral Load
Age of Onset
Immediate-Early Genes
Fatal Outcome
Lymphoproliferative Disorders
Hematopoietic Stem Cell Transplantation
DNA
Lymphocyte Subsets
Cause of Death

ASJC Scopus subject areas

  • Dermatology

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Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites. / Miyake, Tomoko; Yamamoto, T.; Hirai, Youji; Otsuka, M.; Hamada, T.; Tsuji, K.; Morizane, Shin; Suzuki, D.; Aoyama, Y.; Iwatsuki, K.

In: British Journal of Dermatology, Vol. 172, No. 1, 01.01.2015, p. 56-63.

Research output: Contribution to journalArticle

Miyake, Tomoko ; Yamamoto, T. ; Hirai, Youji ; Otsuka, M. ; Hamada, T. ; Tsuji, K. ; Morizane, Shin ; Suzuki, D. ; Aoyama, Y. ; Iwatsuki, K. / Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites. In: British Journal of Dermatology. 2015 ; Vol. 172, No. 1. pp. 56-63.
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T1 - Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites

AU - Miyake, Tomoko

AU - Yamamoto, T.

AU - Hirai, Youji

AU - Otsuka, M.

AU - Hamada, T.

AU - Tsuji, K.

AU - Morizane, Shin

AU - Suzuki, D.

AU - Aoyama, Y.

AU - Iwatsuki, K.

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Y1 - 2015/1/1

N2 - Background Epstein-Barr virus (EBV)-associated T/natural-killer lymphoproliferative disorders form a group of diseases that includes classical and systemic hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). Patients with systemic HV (sHV) and HMB often have a poor prognosis, although little is known about the prognostic factors. Objectives To elucidate the prognostic factors of HV and HMB. Methods We studied clinicopathological manifestations, routine laboratory findings, anti-EBV titres, EBV DNA load and EBV-encoded gene expression, including expression of BZLF1, in 50 patients with classical HV (cHV), sHV, HMB only and HMB with HV (HMB + HV), and further analysed 30 patients who were available for follow-up. Results The median age of disease onset was 5 years (range 1-74). A follow-up study indicated that fatal outcomes were observed in three of eight patients with sHV, two of six patients with HMB only, and two of five patients with HMB + HV. The main causes of death were complications from haematopoietic stem-cell transplantation and multiorgan failure. There were no fatalities among the 11 patients with cHV. Univariate analysis revealed two poor prognostic indicators: (i) onset age > 9 years and (ii) the expression of an EBV-encoded immediate-early gene transcript, BZLF1 mRNA, in the skin lesions (P <0·001 and P = 0·003, respectively). Conclusions No prognostic correlation was observed in EBV-infected lymphocyte subsets, anti-EBV antibody titres or EBV DNA load. Late onset and EBV reactivation are both related to more severe phenotypes of the disease, and thus may predict a poor prognosis.

AB - Background Epstein-Barr virus (EBV)-associated T/natural-killer lymphoproliferative disorders form a group of diseases that includes classical and systemic hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). Patients with systemic HV (sHV) and HMB often have a poor prognosis, although little is known about the prognostic factors. Objectives To elucidate the prognostic factors of HV and HMB. Methods We studied clinicopathological manifestations, routine laboratory findings, anti-EBV titres, EBV DNA load and EBV-encoded gene expression, including expression of BZLF1, in 50 patients with classical HV (cHV), sHV, HMB only and HMB with HV (HMB + HV), and further analysed 30 patients who were available for follow-up. Results The median age of disease onset was 5 years (range 1-74). A follow-up study indicated that fatal outcomes were observed in three of eight patients with sHV, two of six patients with HMB only, and two of five patients with HMB + HV. The main causes of death were complications from haematopoietic stem-cell transplantation and multiorgan failure. There were no fatalities among the 11 patients with cHV. Univariate analysis revealed two poor prognostic indicators: (i) onset age > 9 years and (ii) the expression of an EBV-encoded immediate-early gene transcript, BZLF1 mRNA, in the skin lesions (P <0·001 and P = 0·003, respectively). Conclusions No prognostic correlation was observed in EBV-infected lymphocyte subsets, anti-EBV antibody titres or EBV DNA load. Late onset and EBV reactivation are both related to more severe phenotypes of the disease, and thus may predict a poor prognosis.

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DO - 10.1111/bjd.13411

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