Use of recombinant factor IX in subjects with haemophilia B undergoing surgery

Recombinant human FIX (rFIX) was evaluated in 28 subjects, including 26 with mild, moderate, or severe haemophilia B and two haemophilia B carriers undergoing 36 surgical procedures. Preoperative rFIX dose was highly correlated with postinfusion FIX activity, r=0.61, P=0.0158. Peri‐ and post‐operative estimated blood loss was similar to that expected in non‐haemophilic individuals, and haemostasis was rated as excellent or good in 34 of 35 (97.1%) of the operative procedures. Transfusions were required in five of 36 (13.9%) procedures, including one liver transplantation, and three knee and one hip arthroplasties. Adverse events occurred in 15 of 28 (53.6%) subjects, but there were no perioperative haemorrhages, thromboembolic events, coagulation activation, viral transmission, or inhibitor formation. A transient low‐responding FIX inhibitor developed in one subject preoperatively, but required no change in treatment and resolved 15 months later. Thus, rFIX was found to be safe and effective in achieving haemostasis in subjects with FIX deficiency undergoing surgery.

[1]  C. Kessler,et al.  Human recombinant factor IX: safety and efficacy studies in hemophilia B patients previously treated with plasma-derived factor IX concentrates. , 2001, Blood.

[2]  P. Collins,et al.  Recombinant factor IX (BeneFix®) by adjusted continuous infusion: a study of stability, sterility and clinical experience , 2001, Haemophilia : the official journal of the World Federation of Hemophilia.

[3]  Rodgers,et al.  Immunosuppressive effects of factor IX products: an in vitro study , 1999, Haemophilia : the official journal of the World Federation of Hemophilia.

[4]  J. Rouse,et al.  Biochemical characterization of recombinant factor IX. , 1998, Seminars in hematology.

[5]  P. Garzone,et al.  Clinical evaluation of recombinant factor IX. , 1998, Seminars in hematology.

[6]  B. Nielsen,et al.  Recombinant Factor IX , 1997, Thrombosis and Haemostasis.

[7]  J. Katz,et al.  Factor IX inhibitors and anaphylaxis in hemophilia B. , 1997, Journal of pediatric hematology/oncology.

[8]  N. Key,et al.  Factor IX inhibitors and anaphylaxis in haemophilia B , 1996, Haemophilia : the official journal of the World Federation of Hemophilia.

[9]  M. Ragni,et al.  Parvovirus B19 infection in patients with hemophilia , 1996, Transfusion.

[10]  B. Furie,et al.  A practical guide to the evaluation and treatment of hemophilia. , 1994, Blood.

[11]  S. Emerson,et al.  Transmission of Hepatitis A to Patients with Hemophilia by Factor VIII Concentrates Treated with Organic Solvent and Detergent To Inactivate Viruses , 1994, Annals of Internal Medicine.

[12]  M. Morfini,et al.  The Design and Analysis of Half-Life and Recovery Studies for Factor VIII and Factor IX , 1991, Thrombosis and Haemostasis.

[13]  J. Lusher,et al.  Thrombogenicity associated with factor IX complex concentrates. , 1991, Seminars in hematology.

[14]  U. Delvos,et al.  Reproducibility of one-stage, two-stage and chromogenic assays of factor VIII activity: a multi-center study. , 1991, Thrombosis research.

[15]  D. Brettler,et al.  Prevalence of hepatitis C virus antibody in a cohort of hemophilia patients. , 1990, Blood.

[16]  S. Cinotti,et al.  Single‐dose pharmacokinetics of Factor IX evaluated by model‐independent methods , 1987, European journal of haematology.

[17]  L. Kingsley,et al.  1986 update of HIV seroprevalence, seroconversion, AIDS incidence, and immunologic correlates of HIV infection in patients with hemophilia A and B , 1987 .

[18]  M. Ragni,et al.  Immune hemolysis by isohemagglutinins in factor IX concentrates , 1987, Transfusion.

[19]  L. Kingsley,et al.  1986 update of HIV seroprevalence, seroconversion, AIDS incidence, and immunologic correlates of HIV infection in patients with hemophilia A and B. , 1987, Blood.

[20]  B. Furie,et al.  Expression, purification, and characterization of recombinant gamma-carboxylated factor IX synthesized in Chinese hamster ovary cells. , 1986, The Journal of biological chemistry.

[21]  K. Kurachi,et al.  Isolation and characterization of a cDNA coding for human factor IX. , 1982, Proceedings of the National Academy of Sciences of the United States of America.

[22]  D. Rees,et al.  Molecular cloning of the gene for human anti-haemophilic factor IX , 1982, Nature.

[23]  K. Bauer,et al.  Studies of the prothrombin activation pathway utilizing radioimmunoassays for the F2/F1 + 2 fragment and thrombin--antithrombin complex. , 1982, Blood.

[24]  D. Green,et al.  A More Uniform Measurement of Factor VIII Inhibitors , 1975, Thrombosis and Haemostasis.