hirudin and Hemophilia-A

We describe a 64-year-old male with severe hemophilia A (factor VIII-dependent), acute myocardial infarction (MI) and congestive heart failure (CHF) who underwent successful multi-vessel percutaneous coronary intervention (PCI). The patient was administered factor VIII transfusion to maintain activity levels between 60-80%. Anticoagulation during the PCI procedure was maintained with the direct thrombin inhibitor, bivalirudin. There were no procedural complications and the patient was discharged home the following day. These results suggest that bivalirudin may be used effectively in patients at very high risk of bleeding with enhanced procedural safety.

Topics: Angioplasty, Balloon, Coronary; Anticoagulants; Antithrombins; Blood Component Transfusion; Coronary Angiography; Factor VIII; Hemophilia A; Hirudins; Humans; Male; Middle Aged; Myocardial Infarction; Peptide Fragments; Radiography, Interventional; Recombinant Proteins

Topics: Animals; Blood Coagulation; Factor IX; Factor VIII; Hemophilia A; Hemophilia B; Hirudin Therapy; Hirudins; Humans; Molecular Biology; Recombinant Proteins

Topics: Aged; Anticoagulants; Hemophilia A; Hirudins; Humans; Male; Peptide Fragments; Percutaneous Coronary Intervention; Perioperative Care; Recombinant Proteins; Treatment Outcome

This article presents the case of a patient with severe haemophilia A who underwent successful multivessel percutaneous coronary intervention (PCI). Patients with haemophilia who are diagnosed with coronary artery disease (CAD) and require intervention present a challenge to doctors due to the high risks of bleeding. The patient was administered recombinant factor VIII pre- and post-procedure. Anticoagulation during PCI was maintained with bivalirudin, a thrombin-specific anticoagulant. There were no complications and the patient tolerated the procedure well. This case suggests that bivalirudin as the sole procedural anticoagulant can be safely used in patients with a very high risk of bleeding undergoing PCI.

Topics: Angioplasty, Balloon, Coronary; Anticoagulants; Coronary Angiography; Coronary Artery Disease; Hemophilia A; Hirudins; Humans; Intraoperative Care; Male; Middle Aged; Peptide Fragments; Recombinant Proteins; Treatment Outcome

Topics: Amino Acid Sequence; Blood Coagulation Tests; Factor VIII; Hemophilia A; Hirudins; Humans; Microchemistry; Microcomputers; Molecular Sequence Data; Nephelometry and Turbidimetry

Extensive aggregation of human platelets can be induced by ADP without secondaryaggregation or release of granule contents. This occurs with washed platelets in Tyrode solution containing 0.35% albumin, human fibrinogen, and apyrase, and in platelet-rich, heparin- or hirudin-plasma. Conditions that caused release during ADP-inducedaggregation were-citrate as the anticoagulant in platelet-rich plasma; addition of citrate (11-15 mM) to a suspension of washed platelets, or to hirudin-plasma or heparin-plasma; suspension of platelets in a medium containing magnesium but no calcium;and the presence of trace amounts of thrombin or aggregated gamma globulin in the platelet suspensions. Acetylsalicylic acid, phenylbutazone, or sulfinpyrazone inhibited secondary aggregation and release in all these circumstances. Heparin or hirudin inhibited ADP-INDUCED SECONDARY AGGREGATION AND RELEASE PROMOTED BY TRACES OF THROMBIN. Although fibrinogen is required for ADP-induced primary aggregation, it does not support secondary aggregation and release, provided that it has no clot-promoting activity. The main agent responsible for ADP-induced secondary aggregation and release in human, citrated, platelet-rich plasma appears to be sodium citrate. Suspending washed human platelets in a medium without calcium mimics the effect of citrate.

Topics: Adenosine Diphosphate; Adenosine Triphosphate; Aspirin; Citrates; Fibrinogen; gamma-Globulins; Hemophilia A; Heparin; Hirudins; Humans; Magnesium; Phenylbutazone; Phosphates; Platelet Adhesiveness; Platelet Aggregation; Serum Albumin; Sulfinpyrazone; Thrombin