UNLABELLED: 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.
UNLABELLED: 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.
Authors: S E Stepp; R Dufourcq-Lagelouse; F Le Deist; S Bhawan; S Certain; P A Mathew; J I Henter; M Bennett; A Fischer; G de Saint Basile; V Kumar Journal: Science Date: 1999-12-03 Impact factor: 47.728
Authors: M Aricò; K Nichols; J A Whitlock; R Arceci; R Haupt; U Mittler; T Kühne; A Lombardi; E Ishii; R M Egeler; C Danesino Journal: Br J Haematol Date: 1999-12 Impact factor: 6.998
Authors: S Scappaticci; C Danesino; E Rossi; C Klersy; G M Fiori; R Clementi; V S Russotto; G Bossi; M Aricò Journal: Br J Haematol Date: 2000-10 Impact factor: 6.998
Authors: Natália Rocha Bedran; Román Carlos; Bruno Augusto Benevenuto de Andrade; Ana Paula Silva Bueno; Mário José Romañach; Cristiane Bedran Milito Journal: Head Neck Pathol Date: 2017-11-21