Life-long bleeding diathesis: effect of orthotopic liver transplantation.

Published

Journal Article

Proper regulation of the fibrinolytic system is critical to the prevention of both thrombosis and hemorrhage. Patients with inherited or acquired excess fibrinolysis may have a bleeding tendency, usually characterized by delayed and posttrauma or postoperative bleeding. The liver plays many roles in this regulation, including the synthesis of plasminogen, alpha 2-antiplasmin (alpha 2-AP) and plasminogen activator inhibitor-1 (PAI-1), and the clearance of tissue-plasminogen activator (t-PA). Inherited deficiencies of alpha 2AP, PAI-1 and inherited excess t-PA associated with clinically significant bleeding have been reported. The authors describe a patient with a life-long bleeding diathesis who demonstrated evidence of excess t-PA. One of two daughters also had a bleeding tendency and demonstrated excess t-PA. The patient developed cirrhosis and underwent orthotopic liver transplantation. Following transplantation, all fibrinolytic parameters returned to normal, and the bleeding diathesis appeared to no longer exist. The effect of liver transplantation on this patient's fibrinolytic abnormalities and the effect of cirrhosis on this patient's laboratory evaluation are discussed.

Full Text

Duke Authors

Cited Authors

  • Humphries, JE; Gonias, SL; Pizzo, SV; Williams, ME

Published Date

  • December 1994

Published In

Volume / Issue

  • 102 / 6

Start / End Page

  • 816 - 820

PubMed ID

  • 7801898

Pubmed Central ID

  • 7801898

International Standard Serial Number (ISSN)

  • 0002-9173

Digital Object Identifier (DOI)

  • 10.1093/ajcp/102.6.816

Language

  • eng

Conference Location

  • England