hirudin and Budd-Chiari-Syndrome

Polycythemia vera is a Philadelphia chromosome-negative myeloproliferative disorder with incidence of 1% under the age of 25. The Budd-Chiari syndrome (BCS) is a well known complication of polycythemia vera even in children, and characterized by occlusion of hepatic outflow. A computerized archive search of medical records at Sheba Medical Center of the past three decades of patients with polycythemia vera and BCS under the age of 25 years was performed. A work-up for JAK2 V617F mutation and thrombophilia was done. Medical charts and imaging tests were carefully reviewed. Three patients under the age of 22 were finally recruited. Two of those were found in life-threatening condition and improved clinically following treatment with bivalirudin, a direct thrombin inhibitor. It is conceivable that bivalirudin contributed to a favorable outcome of those patients in comparison to historical outcome previously reported. In conclusion, polycythemia vera in the young is not a mild disease since BCS, which is one of its complication, can be fatal even in those age group unrelated to the presence of hereditary thrombophilia. Once BCS occurs, we would suggest giving a trial with bivalirudin before an invasive procedure is planned.

Topics: Adolescent; Antithrombins; Budd-Chiari Syndrome; Child; Female; Hirudins; Humans; Janus Kinase 2; Liver; Male; Mutation; Peptide Fragments; Polycythemia Vera; Recombinant Proteins; Thrombin; Thrombophilia; Treatment Outcome; Young Adult

Type II heparin-induced thrombocytopenia (HIT) is an immune-mediated syndrome that may arise in a time-dependent manner following heparin therapy, placing patients at significant risk for thromboembolic events. Therapy includes anticoagulation with a direct thrombin inhibitor and avoidance of heparin. We report a patient with Budd-Chiari syndrome and a history of heparin-induced thrombocytopenia who presented for orthotopic liver transplant and required postoperative anticoagulation with bivalirudin. During the post-transplant graft function improvement, we observed a significant dose-effect alteration manifested by an increased bivalirudin dose requirement as factor V activity increased. This observation is an important consideration in the attempt to maintain an optimal balance between effective anticoagulation and a reduced risk of postoperative bleeding.

Topics: Adult; Anticoagulants; Blood Coagulation; Budd-Chiari Syndrome; Dose-Response Relationship, Drug; Factor V; Heparin; Hirudins; Humans; Liver; Liver Transplantation; Male; Partial Thromboplastin Time; Peptide Fragments; Recombinant Proteins; Thrombocytopenia; Thrombosis

Topics: Adult; Anticoagulants; Antithrombins; Budd-Chiari Syndrome; Cholecystitis; Disease Progression; Drug Resistance; Drug-Related Side Effects and Adverse Reactions; Factor V; Female; Follow-Up Studies; Hirudins; Humans; Infusions, Intravenous; Injections, Subcutaneous; Mutation; Odds Ratio; Recombinant Proteins; Thrombophilia; Treatment Outcome

We report on a patient suffering from Budd-Chiari disease who developed heparin-induced thrombocytopenia preoperatively. Dorsocranial liver resection and hepatoatrial anastomosis were performed with the extracorporeal circulation and perioperative anticoagulation was achieved with r-hirudin. Surprisingly, thrombus formation was observed in the venous reservoir although intraoperative anticoagulation values were within the targeted level. An additional bolus of hirudin and rinsing the reservoir allowed unproblematic discontinuation of the cardiopulmonary bypass.

Topics: Adult; Budd-Chiari Syndrome; Dose-Response Relationship, Drug; Extracorporeal Circulation; Heart Atria; Heparin; Hepatic Veins; Hirudins; Humans; Infusions, Intravenous; Male; Partial Thromboplastin Time; Recurrence; Thrombocytopenia; Venous Thrombosis

Topics: Acenocoumarol; Anticoagulants; Budd-Chiari Syndrome; Endotoxins; Escherichia coli Infections; Heparin; Hirudins; Hyperlipidemias; Pharmacology; Prothrombin Time; Rats; Research; Salmonella Infections; Salmonella Infections, Animal; Thrombophlebitis; Toxicology; Venous Thrombosis