Experience with recombinant factor VIIA in an infant hemophiliac with inhibitors to FVIII:C undergoing emergency central line placement. A case report.
An infant boy with severe factor VIII (FVIII) deficiency hemophilia and antibodies against coagulation FVIII is presented. This case is unique from the point of view of the young age of development and recognition of inhibitors to the FVIII and because this patient received recombinant factor VIIa (rFVIIa), prior to a life-saving surgical procedure. Conventional treatment options had been exhausted. The inhibitor was an IgG antibody that had rapidly increased to 100 Bethesda Units (BU). A broviac central line was placed to provide i.v. access for the purpose of treatment. Porcine FVIII had been used but an inhibitor rapidly developed to that product. Using rFVIIa, hemostasis was secured for placement of a second central line and to stop life-threatening mucosal bleeding. rVIIa was an effective treatment alternative for the management of this patient with inhibitors.
Bell
B A
BA
Department of Pediatric Hematology/Oncology, Atlanta Georgia School of Medicine 30322.
Birch
K
K
Glazer
S
S
D001323:Autoantibodies / Q000276:immunology*; D002405:Catheterization, Central Venous / Q000009:adverse effects; D005169:Factor VIII / Q000276:immunology*; D015942:Factor VIIa / Q000627:therapeutic use*; D006467:Hemophilia A / Q000628:therapy*; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D006472:Oral Hemorrhage / Q000209:etiology / Q000628:therapy; D011994:Recombinant Proteins / Q000627:therapeutic use
D001323:Autoantibodies; D011994:Recombinant Proteins; D005169:Factor VIII; D015942:Factor VIIa