Liver cirrhosis associated with hereditary hemorrhagic telangiectasia: presentation of a case
Girardi, A.; Piazza, I.
Giornale di Clinica Medica 69(1): 37-41
1988
ISSN/ISBN: 0017-0275 PMID: 3384230 Document Number: 308041
Hereditary hemorrhagic teleangiectasia (HHT) is a dominant autosomic disorder characterized by widespread presence of capillary and venous malformations ("teleangiectases") within the skin, mucous membranes and various internal organs. These malformations may cause repeated and even serious haemorrhages (epistaxis, hematemesis, melena, haematuria, and so on). As far as lungs are concerned, sometimes an arterovenous fistula can be observed. The liver gets involved in this pathology more often than it was once thought. In the last thirty years, many authors described the association between HHT and hepatic cirrhosis, suggesting that cirrhosis should be a direct result of HHT, since the presence of teleangiectases in the liver may cause the formation of fibrosis and regenerating nodules by means of the local variations of the blood flow. This association is contested by other authors who emphasize on how often the HHT patients may develop hepatic cyrrhosis due to different causes, even if these are connected with the original disease: hemocromatosis, post-transfusion hepatitis, congestive heart failure. The authors report the case of a male patient (74 years old), affected by a classic form of HHT together with hepatic cirrhosis of unknown cause. The authors suggest that cirrhosis is a rare but well-established result of HHT.