An Unusual Cause of Liver Allograft Loss in a Poorly Compliant Young Adult

Mylène Sebagh1,2,3*, Jean-Luc Taupin4 and Faouzi Saliba2,3,5

1Hôpital Paul-Brousse Ap-Hp, Laboratoire Anatomie Pathologique, France
2Inserm, University of Paris-Saclay, France
3Paris-Sud University, University of Paris-Saclay, France
4Department of Immunology and Histocompatibility, AP-HP Saint-Louis Hospital, France
5Hôpital Paul-Brousse Ap-Hp, Centre Hépato-Biliaire, France

^*Correspondance to: Mylène Sebagh 

 PDF  Full Text DOI: 10.25107/2474-1647.3334

Abstract

Non-adherence to immunosuppressant therapy impairs long-term outcome after Liver Transplantation (LT), especially among pediatric recipients reaching adulthood. The main consequence is the development of chronic rejection. A young man underwent LT for biliary atresia. The follow-up was remarkable by poor adherence to immunosuppressant therapy. The last followup liver biopsy showed chronic rejection and one periportal non-necrotizing epithelioid granuloma that was neglected. The deterioration in the hepatic function due to presumed chronic rejection and the development of chronic kidney disease due to calcineurin inhibitor’s toxicity led to a combined repeat LT and kidney transplantation at 21 years post-LT. Histologic examination of the explanted liver revealed granulomatous liver disease, unique by the surprising location of the granulomas, realizing « granulomatous endothelialitis » of veins responsible for their lumen narrowing. The extensive etiology work-up (including donor-specific anti-HLA antibodies and antibodies against the endothelin-1 receptor type A and the angiotensin II receptor type 1 two of the most potent vasoconstrictors reported to date) remained negative. This de novo case of granulomatous liver disease had unique presentation and ads further evidence that a careful search must be carried out for a cause when granuloma is seen on liver biopsy.

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Cite the article

Sebagh M, Taupin J-L, Saliba F. An Unusual Cause of Liver Allograft Loss in a Poorly Compliant Young Adult. Clin Surg. 2021; 6: 3334..