Lymphomatoid granulomatosis associated with azathioprine therapy in Crohn disease

BMC Gastroenterology

Paul J Belletrutti³  Jay Patel¹  Cameron Griffiths²  William Connors² 

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

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

Others  :  855030
DOI  :  10.1186/1471-230X-14-127

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

【 摘 要 】

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.

【 授权许可】

   
2014 Connors et al.; licensee BioMed Central Ltd.

【 图 表 】

【 参考文献 】

• [1]Liebow AA, Carrington CR, Friedman PJ: Lymphomatoid granulomatosis. Hum Pathol 1972, 3:457-558.
• [2]Jaffe ES, Wilson WH: Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. Cancer Surv 1997, 30:233-248.
• [3]Fauci AS, Haynes BF, Costa J, Katz P, Wolff SM: Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. N Engl J Med 1982, 306:68-74.
• [4]Katzenstein AL, Doxtader E, Narendra S: Lymphomatoid granulomatosis: insights gained over 4 decades. Am J Surg Pathol 2010, 34:e35-e48.
• [5]Colby TV: Current histological diagnosis of lymphomatoid granulomatosis. Mod Pathol 2012, 25:S39-S42.
• [6]Destombe S, Bouron-DalSoglio D, Rougemont AL, Fournet JC, Ovetchkine P, Champagne J, Deslandres C: Lymphomatoid granulomatosis: a unique complication of Crohn disease and its treatment in pediatrics. J Pediatr Gastroenterol Nutr 2010, 50:559-561.
• [7]Katherine Martin L, Porcu P, Baiocchi RA, Erter JW, Chaudhary AR: Primary central nervous system lymphomatoid granulomatosis in a patient receiving azathioprine therapy. Clin Adv Hematol Oncol 2009, 7:65-68.
• [8]Herrinton LJ, Liu L, Weng X, Lewis JD, Hutfless S, Allison JE: Role of thiopurine and anti-TNF therapy in lymphoma in inflammatory bowel disease. Am J Gastroenterol 2011, 106:2146-2153.
• [9]Lewis JD, Bilker WB, Brensinger C, Dern JJ, Vaughn DJ, Strom BL: Inflammatory bowel disease is not associated with an increased risk of lymphoma. Gastroenterology 2001, 121:1080-1087.
• [10]Beaugerie L, Sokol H, Seksik P: Noncolorectal malignancies in inflammatory bowel disease: more than meets the eye. Dig Dis 2009, 27:375-381.
• [11]Kandiel A, Fraser AG, Korelitz BI, Brensinger C, Lewis JD: Increased risk of lymphoma among inflammatory bowel disease patients treated with azathioprine and 6-mercaptopurine. Gut 2005, 54:1121-1125.
• [12]Beaugerie L, Brousse N, Bouvier AM, Colombel JF, Lemann M, Cosnes J, Hebuterne X, Cortot A, Bouhnik Y, Gendre JP, Simon T, Maynadie M, Hermine O, Faivre J, Carrat F, CESAME Study Group: Lymphoproliferative disorders in patients receiving thiopurines for inflammatory bowel disease: a prospective observational cohort study. Lancet 2009, 374:1617-1625.
• [13]Dayharsh GA, Loftus EV Jr, Sandborn WJ, Tremaine WJ, Zinsmeister AR, Witzig TE, Macon WR, Burgart LJ: Epstein-Barr virus-positive lymphoma in patients with inflammatory bowel disease treated with azathioprine or 6-mercaptopurine. Gastroenterology 2002, 122:72-77.