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

Abstract

Summary 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.

A patient with elderly-onset atypical hydroa vacciniforme with an indolent clinical course

“…This term is applicable to a group with an indolent clinical course, as in our patient. Miyake et al . reported that an onset age of > 9 years is one of the poor prognostic indicators of EBV + LPD and Kimura et al .…”

“…Elderly onset is extremely rare, and the clinical features of this patient population remain unclear. Although the prognostic factors of EBV‐associated LPD have been controversial, it is known that late onset is closely associated with severe phenotype in EBV‐associated LPD . Here, we describe a patient with elderly‐onset atypical HV.…”

Epstein-Barr Virus–Associated Lymphoproliferative Disorders: Review and Update on 2016 WHO Classification

“…Severe HV-type tends to exhibit more extensive skin lesions and systemic manifestations of fever, hepatomegaly, serologic abnormalities, and peripheral NK lymphocytosis. The severe type of HV often progresses to EBV-associated NK/T-cell malignancy [22,23]. …”

Risk of mortality in hydroa vacciniforme and hypersensitivity to mosquito bites

“…In this issue of BJD , Miyake et al . further define this spectrum of cHV, systemic HV (sHV) and HMB.…”