Recurrence of hereditary hemorrhagic telangiectasia after liver transplantation: clinical implications and physiopathological insights.

Dumortier J1, Dupuis-Girod S2, Valette PJ3, Valent A4, Guillaud O1, Saurin JC5, Hervieu V6, Robinson P7, Plauchu H2, Paliard P5, Boillot O1, Scoazec JY4,6.

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Abstract

Liver transplantation (LT) has been proposed as a curative treatment in hereditary hemorrhagic telangiectasia (HHT) with severe hepatic involvement. We provide a long-term evaluation of graft status after LT for HHT, with a focus on the risk of recurrence. The present study included all patients prospectively followed-up after LT for HHT in the Lyon liver transplant unit from 1993 to 2010, with a survival of more than 1 year. Protocol clinical, radiological, and histological examinations were performed at regular intervals. Fourteen patients were included (13 women and 1 man). Median age at LT was 52.5 years (range [33.1-66.7]). In 8 patients (7 female), disease recurrence was diagnosed by abnormal radiological features, suggestive of microcirculatory disturbances. Typical vascular lesions, including telangiectasia, were demonstrated by liver biopsy in 5 of these patients. The median interval between LT and diagnosis of recurrence was 127 months (range [74-184]). The risk of recurrence increased over time; estimated cumulative risk was 47.9% at 15 years. Liver tissue analysis found the coexistence of an angiogenic process combined with endothelial microchimerism, as shown by the presence of vascular lining cells of recipient origin. In conclusion, the present data show that disease recurrence occurs, usually after a long delay, in a significant number of patients treated by LT for liver complications of HHT. This strongly supports the necessity of a life-long follow-up and suggests that therapeutic strategy needs discussion, and evaluation, especially the role of potential adjuvant treatments to LT, such as anti-angiogenic medications, when recurrent disease appears. This article is protected by copyright. All rights reserved.