title: Hemophagocytic lymphohistiocytosis caused by primary Epstein-Barr virus in patient with Crohn's disease.
abstract: We present a case of a 19-year-old man with a 6-year history of Crohn's disease (CD), previously treated with 6-mercaptopurine, who was admitted to our department for Epstein-Barr virus (EBV) infection and subsequently developed a hemophagocytic lymphohistiocytosis (HLH). HLH is a rare disease which causes phagocytosis of all bone marrow derived cells. It can be a primary form as a autosomic recessive disease, or a secondary form associated with a variety of infections; EBV is the most common, the one with poorer prognosis. The incidence of lymphoproliferative disorders was increased in patients with inflammatory bowel disease (IBD) treated with thiopurines. Specific EBV-related clinical and virological management should be considered when treating a patient with IBD with immunosuppressive therapy. Moreover EBV infection in immunosuppressed patient can occur with more aggressive forms such as encephalitis and diffuse large B cell lymphoma. Our case confirms what is described in the literature; patients with IBD, particularly patients with CD receiving thiopurine therapy, who present 5 d of fever and cervical lymphadenopathy or previous evidence of lymphopenia should be screened for HLH.
authors: Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom.
authors: Francesco
authors: F
authors: Virdis
authors: Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom.
authors: Sara
authors: S
authors: Tacci
authors: Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom.
authors: Federico
authors: F
authors: Messina
authors: Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom.
authors: Massimo
authors: M
authors: Varcada
keywords: Crohn’s disease; Epstein-Barr virus infection; Hemophagocytic lymphohistiocytosis; Immunosupressive therapy; Thiopurines