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.
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.
Authors: Rita Alaggio; Catalina Amador; Ioannis Anagnostopoulos; Ayoma D Attygalle; Iguaracyra Barreto de Oliveira Araujo; Emilio Berti; Govind Bhagat; Anita Maria Borges; Daniel Boyer; Mariarita Calaminici; Amy Chadburn; John K C Chan; Wah Cheuk; Wee-Joo Chng; John K Choi; Shih-Sung Chuang; Sarah E Coupland; Magdalena Czader; Sandeep S Dave; Daphne de Jong; Ming-Qing Du; Kojo S Elenitoba-Johnson; Judith Ferry; Julia Geyer; Dita Gratzinger; Joan Guitart; Sumeet Gujral; Marian Harris; Christine J Harrison; Sylvia Hartmann; Andreas Hochhaus; Patty M Jansen; Kennosuke Karube; Werner Kempf; Joseph Khoury; Hiroshi Kimura; Wolfram Klapper; Alexandra E Kovach; Shaji Kumar; Alexander J Lazar; Stefano Lazzi; Lorenzo Leoncini; Nelson Leung; Vasiliki Leventaki; Xiao-Qiu Li; Megan S Lim; Wei-Ping Liu; Abner Louissaint; Andrea Marcogliese; L Jeffrey Medeiros; Michael Michal; Roberto N Miranda; Christina Mitteldorf; Santiago Montes-Moreno; William Morice; Valentina Nardi; Kikkeri N Naresh; Yasodha Natkunam; Siok-Bian Ng; Ilske Oschlies; German Ott; Marie Parrens; Melissa Pulitzer; S Vincent Rajkumar; Andrew C Rawstron; Karen Rech; Andreas Rosenwald; Jonathan Said; Clémentine Sarkozy; Shahin Sayed; Caner Saygin; Anna Schuh; William Sewell; Reiner Siebert; Aliyah R Sohani; Reuben Tooze; Alexandra Traverse-Glehen; Francisco Vega; Beatrice Vergier; Ashutosh D Wechalekar; Brent Wood; Luc Xerri; Wenbin Xiao Journal: Leukemia Date: 2022-06-22 Impact factor: 12.883
Authors: Z Peng; W W Xu; Y Sham; H Lam; D Sun; L Cheng; N F Rasic; Q Guan; A A James; F E R Simons Journal: Allergy Date: 2016-03-06 Impact factor: 13.146
Authors: Jeffrey I Cohen; Irini Manoli; Kennichi Dowdell; Tammy A Krogmann; Deborah Tamura; Pierce Radecki; Wei Bu; Siu-Ping Turk; Kelly Liepshutz; Ronald L Hornung; Hiva Fassihi; Robert P Sarkany; Lori L Bonnycastle; Peter S Chines; Amy J Swift; Timothy G Myers; Melissa A Levoska; John J DiGiovanna; Francis S Collins; Kenneth H Kraemer; Stefania Pittaluga; Elaine S Jaffe Journal: Blood Date: 2019-05-07 Impact factor: 25.476
Authors: Se Jin Oh; Jongeun Lee; Ji-Hye Park; Jong Hee Lee; Junhun Cho; Young-Hyeh Ko; Dongyoun Lee Journal: Ann Dermatol Date: 2021-05-04 Impact factor: 1.444