ABSTRACT
We describe the experience of a single medical centre with continuous factor VIII (FVIII) infusion therapy in a cohort of patients undergoing elective surgery. Twenty-eight patients had a total of 45 procedures. Intraoperative haemostasis was considered excellent in all 45 cases. FVIII levels were maintained between 46% and 191% of normal (median, 103%) for 2-7 days. Bleeding occurred after five procedures (11%) at times when factor VIII levels were within haemostatic range. No patient required reoperation to control bleeding. There were no cases of sepsis related to continuous infusion of factor VIII. We conclude that continuous infusion: (1) is a safe and effective means of replacement therapy in patients with haemophilia undergoing surgery; (2) provides easier monitoring and more constant coagulation factor levels; and (3) has the potential to decrease the cost of replacement therapy by reducing overall usage of product.
Subject(s)
Factor VIII/administration & dosage , Hemophilia A/drug therapy , Hemostasis, Surgical/methods , Adolescent , Adult , Aged , Child , Child, Preschool , Factor VIII/analysis , Hemophilia A/blood , Hemophilia A/surgery , Humans , Infant , Infusions, Intravenous , Length of Stay , Male , Middle Aged , Postoperative Hemorrhage/prevention & control , Postoperative Hemorrhage/therapy , Recombinant Proteins/administration & dosage , Retrospective StudiesABSTRACT
This is the first published report documenting the successful use of intrauterine infusion of factor VIII (FVIII) in order to reduce the risk of intracranial haemorrhage in a foetus with documented haemophilia A. This approach provides another option for management of newborns with documented coagulation factor deficiency. The subsequent development of an inhibitor directed against FVIII is believed to be related to exposure to exogenous FVIII in the presence of an inversion mutation and not to the intrauterine procedure.
Subject(s)
Factor VIII/administration & dosage , Fetal Diseases/drug therapy , Hemophilia A/drug therapy , Blood Transfusion, Intrauterine , Female , Hemophilia A/complications , Humans , Infusions, Parenteral , Intracranial Hemorrhages/prevention & control , Male , PregnancyABSTRACT
We describe a patient with mild haemophilia B who developed symptomatic venous thromboembolism after hip arthroplasty for a traumatic fracture. A deep vein thrombosis developed in the operated leg while he was receiving a high-purity factor IX concentrate. Subsequently, he was determined to be a heterozygous carrier for the factor V Arg506Gln (Leiden) mutation. This case illustrates the importance of providing thromboprophylaxis for all patients with haemophilia receiving coagulation factor replacement and who undergo surgical procedures known to be associated with a high risk of venous thromboembolism. In patients with haemophilia and a family history of venous thromboembolism, preoperative screening for the presence of the factor V Arg506Gln mutation and other thrombophilias may be useful.
