Thrombelastographic evidence of hyperfibrinolysis during liver transplantation for familial amyloidotic polyneuropathy ATTR met 30.

FAMILIAL amyloidotic polyneuropathy (FAP) ATTR Met 30 is the result of an inherited disorder of transthyretin metabolism. Both amyloid transthyretin and normal transthyretin are produced in the liver and the transplantation is a successful therapy for this disease and is performed in spite of the completely normal liver function of these patients. A high incidence of hyperfibrinolysis was reported during liver transplantation but these studies were performed in patients with previous liver insufficiency and the relative role of preoperative factors (liver disease, coagulation disorders, increased fibrinolytic activity) and intraoperative factors (surgical trauma, hypotension, and graft reperfusion) is not clearly established. As far as we know, the incidence and the severity of hyperfibrinolysis during liver transplantation in patients without preoperative liver insufficiency has not yet been studied. The aim of this study was to evaluate the incidence of hyperfibrinolysis during liver transplantation in FAP and to correlate these findings with several perioperative factors.