SNFGE SNFGE
 
Thématique :
- Foie
Originalité :
Très original
Solidité :
A confirmer
Doit faire évoluer notre pratique :
Dans certains cas
 
 
Nom du veilleur :
Professeur Christine SILVAIN
Coup de coeur :
 
 
Hepatology
  2019/05  
 
  2019 May;69(5):2232-2240.  
  doi: 10.1002/hep.30424.  
 
  Recurrence of Hereditary Hemorrhagic Telangiectasia After Liver Transplantation: Clinical Implications and Physiopathological Insights.  
 
  Dumortier J, Dupuis-Girod S, Valette PJ, Valent A, Guillaud O, Saurin JC, Hervieu V, Robinson P, Plauchu H, Paliard P, Boillot O, Scoazec JY  
  https://www.ncbi.nlm.nih.gov/pubmed/30549294  
 
 

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 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.

 

 
Question posée
 
Quel est le risque de récidive hépatique de la maladie de Rendu-Osler après transplantation hépatique (TH) ?
 
Question posée
 
Dans cette petite série française mais la seule existante, le risque cumulé de récidive augmente avec le temps estimé à 48% à 15 ans.
 
Commentaires

Très beau travail confirmant la nécessité de discuter dans un centre de référence de l’indication de TH pour cette maladie rare et de ne pas passer à côté des traitements anti-angiogéniques avant la TH.

 
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