Liver Transplantation for Prothrombin Deficiency Causing Life-Threatening Bleeds.

We report here the first case of living-related liver transplantation to correct a severe bleeding diathesis. A Caucasian infant was diagnosed at 11 weeks old with prothrombin deficiency after a spontaneous subdural hemorrhage. Even though he had a Factor II activity level of 0.08, he had severe clinical complications including umbilical bleeding, hemorrhagic shock after circumcision and bleeding per rectum.

Prophylactic treatment with prothrombin complex concentrate prevented further bleeding episodes. Unfortunately, he developed clots of the superior vena cava and of the inferior vena cava in the context of central venous catheters. On-demand therapy via peripheral IV was followed for almost a year.

He then had severe subdural and intraparenchymal hemorrhages. His clinical course did not correlate with measured Factor II activity. Investigations looking for other coagulation disorders were negative.

He underwent a living-related liver transplant from his father in order to correct the bleeding diathesis. After transplant his Factor II level rose to 0.51. He required a second liver transplant (cadaveric) and died from tranplant related complications within a month.

Management decisions must reflect the clinical severity of bleeding and not the activity level in rare bleeding disorders where no treatment guidelines exist.