Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites.
Br J Dermatol
; 172(1): 56-63, 2015 Jan.
Article
em En
| MEDLINE
| ID: mdl-25234411
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Infecções por Vírus Epstein-Barr
/
Hidroa Vaciniforme
/
Hipersensibilidade
/
Mordeduras e Picadas de Insetos
/
Culicidae
Idioma:
En
Ano de publicação:
2015
Tipo de documento:
Article