Year 2018 / Volume 110 / Number 6
Letter to the Editor
Idiopathic portal hypertension with regard to thiopurine treatment

409

DOI: 10.17235/reed.2018.5256/2017

Salvador Machlab, Mireia Miquel, Mercedes Vergara,

Abstract
Idiopathic portal hypertension (IPH) is an infrequent adverse reaction to the use of thiopurines that tends to be overlooked. Herein, we present a patient with ileocolic Crohn's disease treated with azathioprine who presented ascites, esophageal varices and splenomegaly without any signs of liver cirrhosis. A portal hemodynamics study revealed a normal portosystemic gradient compatible with presinusoidal portal hypertension. Finally, IPH was diagnosed after a liver biopsy. IPH secondary to thiopurines is due to a 6-thioguanine nucleotide (6-TGN)-dependent reaction and occurs predominantly between three months and three years after the start of treatment. The onset is usually insidious and thrombocytopenia is the first manifestation. The definitive diagnosis is obtained by liver biopsy.
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References
1.Suárez Ferrer C, Llop Herrera E, Calvo Moya M, et al. Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease. Rev Esp Enferm Dig. 2016;108(2):79-83
2. Calabrese E, Hanauer SB. Assessment of non-cirrhotic portal hypertension associated with thiopurine therapy in inflammatory bowel disease. J Crohn’s Colitis 2011; 5: 48-53.
3. Seiderer J, Zech CJ, Diebold J, et al. Nodular regenerative hyperplasia: a reversible entity associated with azathioprine therapy. Eur J Gastroenterol Hepatol 2006; 18: 553-5.
4. Gilissen LPL, Derijks LJJ, Driessen A, et al. Toxicity of 6-thioguanine: no hepatotoxicity in a series of IBD patients treated with long-term, low dose 6-thioguanine. Dig Liver Dis 2007; 39: 156-159.
5. Jharap B, van Asseldonk DP, de Boer NKH, et al. Diagnosing Nodular Regenerative Hyperplasia of the Liver Is Thwarted by Low Interobserver Agreement. PLoS One 2015; 10: e0120299.
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Machlab S, Miquel M, Vergara M. Idiopathic portal hypertension with regard to thiopurine treatment. 5256/2017


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Publication history

Received: 12/09/2017

Accepted: 27/01/2018

Online First: 16/04/2018

Published: 31/05/2018

Article Online First time: 216 days

Article editing time: 261 days


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