HETEROZYGOUS PROTEIN-C DEFICIENCY AND DYSFIBRINOGENEMIA ACQUIRED BY LIVER-TRANSPLANTATION

Orthotopic liver transplantation is now a successful treatment for end -stage liver diseases. Since most components of the coagulation system are synthesized by liver parenchymal cells, there is always a risk of genetic defects of hemostasis being transmitting by liver transplanta tion. Some coagulation factor defects, such as protein C deficiency, d o not induce abnormalities in routine coagulation tests and, thus, go undetected before organ procurement. We report the first case, to our knowledge, of the transmission of heterozygous protein C deficiency, a n autosomal recessive genetic defect, associated with dysfibrinogenemi a, an autosomal dominant trait, by liver transplantation. Both the rec ipient and the donor presented with severe thrombotic complications. T his case shows that potentially morbid genetic defects can be transmit ted by organ transplantation, and it emphasizes the difficulty associa ted with organ procurement criteria, particularly for liver transplant ation, in which routine blood tests appear insufficient for determinin g whether or not organs can or should be procured from a given donor.