title: Total thrombin-activatable fibrinolysis inhibitor (TAFI) antigen and pro-TAFI in patients with haemophilia A.
abstract: Pro-thrombin-activatable fibrinolysis inhibitor (pro-TAFI), also known as TAFI, procarboxypeptidase U, or procarboxypeptidase B, is a relatively recently described plasma glycoprotein synthesized in the liver. It can be catalysed into its active form, TAFI (TAFIa, carboxypeptidase U or B) by a complex of thrombin/thrombomodulin. TAFI can potentially inhibit fibrinolysis by removing carboxyterminal lysine residues from partially degraded fibrin, decreasing plasminogen binding on the surface of fibrin, which thereby results in a decrease of the fibrinolytic activity. As TAFI represents a connection between coagulation and fibrinolysis, it can be expected that TAFI levels are altered in different thrombotic and haemorrhagic diseases, such as haemophilia A. Total TAFI antigen (including pro-TAFI, TAFI and the inactive form of TAFI [TAFIi]) and pro-TAFI were determined in 17 patients with haemophilia A. Thirteen healthy age-matched volunteers served as controls. No significant difference in levels of total TAFI antigen was observed between controls and patients with haemophilia, although it was slightly decreased in patients with haemophilia. Pro-TAFI was significantly reduced in haemophilia patients compared to controls (P=0.0113). TAFI antigen levels similar to controls have already been described in different clinical conditions, including haemophilia A. Decrease of pro-TAFI in haemophilia A can be an additional factor, together with decrease in thrombin generation, which induces impaired activation of pro-TAFI to TAFI, and could cause accelerated fibrinolysis. This supports the validity of usage of antifibrinolytics in the treatment of haemophilia A. In this paper we use new nomenclature for TAFI, and we believe that this recommended terminology for different forms of TAFI can simplify further standardization in TAFI investigation.
authors.affiliation: Coagulation Research, Department of Surgical Sciences, Karolinska Institutet, Karolinska Hospital, Stockholm, Sweden. jovan.antovic@kirurgi.ki.se
authors.forename: J
authors.initials: J
authors.lastname: Antovic
authors.forename: S
authors.initials: S
authors.lastname: Schulman
authors.forename: A
authors.initials: A
authors.lastname: Eelde
authors.forename: M
authors.initials: M
authors.lastname: Blombäck
mesh_terms: D000328:Adult; D000368:Aged; D025901:Carboxypeptidase B2 / Q000097:blood*; D016022:Case-Control Studies; D004792:Enzyme Precursors / Q000097:blood*; D006467:Hemophilia A / Q000097:blood* / Q000201:enzymology; D006801:Humans; D007118:Immunoassay; D008875:Middle Aged; D009626:Terminology as Topic
chemical_list: D004792:Enzyme Precursors; D025901:Carboxypeptidase B2