Recurrence of Hereditary Hemorrhagic Telangiectasia After Liver Transplantation: Clinical Implications and Physiopathological Insights
| Autor: | Pierre Paliard, Jérôme Dumortier, Pierre-Jean Valette, Alexander Valent, Sophie Dupuis-Girod, Olivier Guillaud, Olivier Boillot, Jean-Yves Scoazec, Valérie Hervieu, Henri Plauchu, Jean-Christophe Saurin, Philip Robinson |
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| Rok vydání: | 2019 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty medicine.medical_treatment Transplants Disease Liver transplantation Gastroenterology 03 medical and health sciences Postoperative Complications 0302 clinical medicine Recurrence Internal medicine Liver tissue medicine Recurrent disease Humans Telangiectasia Aged Hepatology medicine.diagnostic_test business.industry Microchimerism Middle Aged Liver Transplantation Hepatic Involvement 030104 developmental biology Liver Liver biopsy Female Telangiectasia Hereditary Hemorrhagic 030211 gastroenterology & hepatology France medicine.symptom business Follow-Up Studies |
| Zdroj: | Hepatology. 69:2232-2240 |
| ISSN: | 1527-3350 0270-9139 |
| DOI: | 10.1002/hep.30424 |
| Popis: | 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 one man). Median age at LT was 52.5 years (range: 33.1-66.7). In eight patients (seven female), disease recurrence was diagnosed by abnormal radiological features, suggestive of microcirculatory disturbances. Typical vascular lesions, including telangiectasia, were demonstrated by liver biopsy in five 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. 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 lifelong follow-up and suggests that therapeutic strategy needs discussion and evaluation, especially of the role of potential adjuvant treatments to LT, such as antiangiogenic medications, when recurrent disease appears. |
| Databáze: | OpenAIRE |
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