European Journal of Pediatrics

, Volume 163, Issue 9, pp 536–539

Identical twin brothers concordant for Langerhans’ cell histiocytosis and discordant for Epstein-Barr virus-associated haemophagocytic syndrome

  • Chun-Jung Chen
  • Tsyr-Yuh Ho
  • Jang-Jih Lu
  • Lai-Fa Sheu
  • Shih-Yi Lee
  • Chiung-Hsi Tien
  • Shin-Nan Cheng
Original Paper

DOI: 10.1007/s00431-004-1493-y

Cite this article as:
Chen, C., Ho, T., Lu, J. et al. Eur J Pediatr (2004) 163: 536. doi:10.1007/s00431-004-1493-y

Abstract

We report on identical twin brothers, one of whom presented at 14 months of age with fever and clinical, laboratory and histological evidence of Epstein-Barr virus-associated haemophagocytic syndrome (EBV-AHS) and 4 months later with typical signs and symptoms of Langerhans’ cell histiocytosis (LCH). The other twin, without previous symptoms, also displayed at that time LCH associated with signs of recent EBV infection, but without symptoms of haemophagocytic syndrome. No mutation in the SH2D1A gene, as observed in X-linked lymphoproliferative disease, or in the perforin gene as observed in some cases of hereditary haemophagocytic syndrome, was found. Conclusion:the occurrence of haemophagocytic syndrome and Langerhans’ cell histiocytosis, although genetically based, can be triggered by environmental agents and viruses, in particular Epstein-Barr virus.

Keywords

Epstein-Barr virusHaemophagocytic syndromeLangerhans’ cell histiocytosisMonozygotic twins

Abbreviations

CAEBV

chronic active Epstein-Barr virus infection

EBV

Epstein-Barr virus

EBV-AHS

EBV-associated haemophagocytic syndrome

HS

haemophagocytic syndrome

HHS

hereditary haemophagocytic syndrome

XLP

X-linked lymphoproliferative disease

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Chun-Jung Chen
    • 1
  • Tsyr-Yuh Ho
    • 1
  • Jang-Jih Lu
    • 2
  • Lai-Fa Sheu
    • 3
  • Shih-Yi Lee
    • 2
  • Chiung-Hsi Tien
    • 1
  • Shin-Nan Cheng
    • 1
  1. 1.Department of Paediatrics, Tri-Service General HospitalNational Defence Medical CentreNeihu, Taipei Taiwan
  2. 2.Division of Clinical PathologyTri-Service General Hospital, National Defence Medical CentreTaipei Taiwan
  3. 3.Department of PathologyTri-Service General Hospital, National Defence Medical CentreTaipei Taiwan