Fibrinogen New Orleans: hereditary dysfibrinogenemia with an A alpha chain abnormality

Abstract

An abnormal fibrinogen (Fibrinogen New Orleans, or FNO) has been found in a 30 year-old woman, her mother and daughter, but not her father. The propositus suffered mild bleeding, but not thrombo-embolism or abnormal wound healing. Plasma and purified fibrinogen from the propositus caused a prolongation in the clotting time of normal plasma. FNO had increased anodal migration when studied by immunoelectrophoresis. We have been able to follow the release of fibrinopeptides A and B using sensitive SDS-PAGE. In FNO, release of the A peptide was markedly delayed, whereas the cleavage of B peptide was much less delayed, compared to that of A peptide.