Biomaterial Database

Surgery in hemophilia A patients with factor VIII inhibitor: 10-year experience. 1996

R Scharf, and W Kucharski, and T Nowak

Department of Surgery, Institute of Hematology and Blood Transfusion, 5 Chocimska Street, 00-957 Warsaw, Poland.

Patients with hemophilia A and circulating anticoagulant (factor VIII inhibitor) present a difficult, even unsolvable problem, particularly if they require surgical treatment and the inhibitor titer is high. During the 1986-1995 period 29 surgical procedures on inhibitor hemophilia A patients were performed in our center. Each of the cases had an individual character, and all demanded special clinical treatment. Based on this experience we present the possibilities of hemostasis maintenance during the perioperative period with high doses of human or porcine factor VIII, aPCC, plasmapheresis, and extracorporeal antibody adsorption to protein A-Sepharose. In some patients hemostasis maintenance requires combined treatment. To induce immunotolerance in patients with inhibitor is the gold standard treatment because it is then possible to achieve proper hemostasis after factor VIII infusion. Various methods of immunotolerance induction have been discussed and compared with our experience with immunotolerance induction in 11 patients with small factor VIII doses (25 IU/kg twice a week) and the modified Malmö protocol in 15 patients.

UI	MeSH Term	Description	Entries
D007108	Immune Tolerance	The specific failure of a normally responsive individual to make an immune response to a known antigen. It results from previous contact with the antigen by an immunologically immature individual (fetus or neonate) or by an adult exposed to extreme high-dose or low-dose antigen, or by exposure to radiation, antimetabolites, antilymphocytic serum, etc.	Immunosuppression (Physiology),Immunosuppressions (Physiology),Tolerance, Immune
D010951	Plasma Exchange	Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions.	Exchange, Plasma,Exchanges, Plasma,Plasma Exchanges
D010956	Plasmapheresis	Procedure whereby plasma is separated and extracted from anticoagulated whole blood and the red cells retransfused to the donor. Plasmapheresis is also employed for therapeutic use.	Double Filtration Plasmapheresis,Therapeutic Immunoadsorption,Therapeutic Plasma Adsorption,Therapeutic Plasmapheresis,Adsorption, Therapeutic Plasma,Adsorptions, Therapeutic Plasma,Double Filtration Plasmaphereses,Filtration Plasmapheresis, Double,Immunoadsorption, Therapeutic,Plasma Adsorption, Therapeutic,Plasmaphereses,Plasmapheresis, Double Filtration,Plasmapheresis, Therapeutic,Therapeutic Immunoadsorptions,Therapeutic Plasma Adsorptions,Therapeutic Plasmaphereses
D003429	Cross Reactions	Serological reactions in which an antiserum against one antigen reacts with a non-identical but closely related antigen.	Cross Reaction,Reaction, Cross,Reactions, Cross
D005169	Factor VIII	Factor VIII of blood coagulation. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation and this action is markedly enhanced by small amounts of thrombin.	Coagulation Factor VIII,Factor VIII Clotting Antigen,Factor VIII Coagulant Antigen,Factor VIII Procoagulant Activity,Thromboplastinogen,Blood Coagulation Factor VIII,F VIII-C,Factor 8,Factor 8 C,Factor Eight,Factor VIIIC,Hyate-C,Hyatt-C,F VIII C,Hyate C,HyateC,Hyatt C,HyattC
D006467	Hemophilia A	The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.	Factor VIII Deficiency,Hemophilia,Autosomal Hemophilia A,Classic Hemophilia,Deficiency, Factor VIII,Factor 8 Deficiency, Congenital,Factor VIII Deficiency, Congenital,Haemophilia,Hemophilia A, Congenital,Hemophilia, Classic,As, Autosomal Hemophilia,Autosomal Hemophilia As,Classic Hemophilias,Congenital Hemophilia A,Congenital Hemophilia As,Hemophilia A, Autosomal,Hemophilia As,Hemophilia As, Autosomal,Hemophilia As, Congenital,Hemophilias, Classic
D006488	Hemostasis, Surgical	Control of bleeding during or after surgery.	Surgical Hemostasis,Hemostases, Surgical,Surgical Hemostases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189	Retrospective Studies	Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.	Retrospective Study,Studies, Retrospective,Study, Retrospective
D013514	Surgical Procedures, Operative	Operations carried out for the correction of deformities and defects, repair of injuries, and diagnosis and cure of certain diseases. (Taber, 18th ed.).	Surgical Procedures,Ghost Surgery,Operative Procedures,Operative Surgical Procedure,Operative Surgical Procedures,Procedure, Operative Surgical,Procedures, Operative Surgical,Surgery, Ghost,Surgical Procedure, Operative,Operative Procedure,Procedure, Operative,Procedure, Surgical,Procedures, Operative,Procedures, Surgical,Surgical Procedure