Case Report
Copyright ©2013 Baishideng Publishing Group Co., Limited. All rights reserved.
World Journal of Gastrointestinal Surgery. Nov 27, 2013; 5(11): 306-308
Published online Nov 27, 2013. doi: 10.4240/wjgs.v5.i11.306
Hemophagocytic lymphohistiocytosis caused by primary Epstein-Barr virus in patient with Crohn’s disease
Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada
Francesco Virdis, Sara Tacci, Federico Messina, Massimo Varcada, Department of Emergency General Surgery, Royal Free Hospital, London, Greater London NW3 2QG, United Kingdom
Author contributions: Virdis F and Varcada M designed the report; Virdis F, Tacci S, Messina F and Varcada M were attending doctors for the patients; Virdis F, Tacci S and Messina F organized the report; and Virdis F wrote paper.
Correspondence to: Francesco Virdis, MD, Department of Emergency General Surgery, Royal Free Hospital, Pond Street, London, Greater London NW3 2QG, United Kingdom. francesco.virdis@nhs.net
Telephone: +44-07-851081406 Fax: +44-02-074726895
Received: August 29, 2013
Revised: September 17, 2013
Accepted: October 18, 2013
Published online: November 27, 2013
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.

Keywords: Crohn’s disease, Hemophagocytic lymphohistiocytosis, Epstein-Barr virus infection, Immunosupressive therapy, Thiopurines

Core tip: About the case we’re presenting, a literature review showed how this rare disease is often lethal and how low is the percentage of patients who have successful treatment. We show our case history and our management which has permitted to discharge the patient with disease regression.