Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease

BMC Gastroenterology

Case Report

William Connors¹  Cameron Griffiths¹  Jay Patel²  Paul J Belletrutti³ 

[1] Department of Medicine, University of Calgary, Calgary, Canada;Department of Pathology, Foothills Medical Centre, Calgary, Canada;Division of Gastroenterology and Hepatology, University of Calgary, Foothills Medical Centre, Calgary, Canada;

关键词: Lymphoproliferative disorders;    Crohn disease;    Inflammatory bowel disease;    Azathioprine;    Thiopurines;   

DOI  :  10.1186/1471-230X-14-127

 received in 2014-04-22, accepted in 2014-06-26,  发布年份 2014

来源: Springer

【 摘 要 】

BackgroundLymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus-associated lymphoproliferative disorder. It most often occurs in patients with immunodeficiency and the clinical course ranges from indolent behavior to that of an aggressive malignancy. Pulmonary, central nervous system and dermatological manifestations are most common. To our knowledge this is the first reported case of LYG related to azathioprine therapy in Crohn disease.Case presentationA twenty-six year old Caucasian woman with colonic Crohn disease on maintenance azathioprine therapy presented with right upper quadrant pain and fever. Diagnostic imaging revealed extensive liver, pulmonary and cerebral lesions. A diagnosis of LYG was made based on the pattern of organ involvement and the immunohistochemical features on liver and lung biopsy.ConclusionsThiopurine therapy for inflammatory bowel disease is associated with an increased incidence of lymphoproliferative disorders. This report highlights the diagnostic challenges associated with LYG. As long-term thiopurine therapy remains central to the management of inflammatory bowel diseases it is essential that both patients and clinicians are aware of this potential adverse outcome.

【 授权许可】

Unknown   
© Connors et al.; licensee BioMed Central Ltd. 2014. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.