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Open Access Case report

Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease

William Connors1, Cameron Griffiths1, Jay Patel2 and Paul J Belletrutti3*

Author Affiliations

1 Department of Medicine, University of Calgary, Calgary, Canada

2 Department of Pathology, Foothills Medical Centre, Calgary, Canada

3 Division of Gastroenterology and Hepatology, University of Calgary, Foothills Medical Centre, Calgary, Canada

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BMC Gastroenterology 2014, 14:127  doi:10.1186/1471-230X-14-127

Published: 14 July 2014

Abstract

Background

Lymphomatoid 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 presentation

A 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.

Conclusions

Thiopurine 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.

Keywords:
Lymphoproliferative disorders; Crohn disease; Inflammatory bowel disease; Azathioprine; Thiopurines