Management of spontaneous inhibitors in children with porcine factor VIII.
Acquired factor VIII (FVIII) inhibitors are rare, typically occurring in the postpartum period or in the elderly. Their occurrence in childhood is distinctly unusual. Acquired FVIII inhibitors are often life-threatening and refractory to treatment with high doses of human FVIII concentrate. Alternative strategies for control of haemostasis include the use of products with FVIII "bypassing" activity or porcine FVIII (pFVIII) concentrate if the pFVIII titre is sufficiently low (<10-20 porcine Bethesda Units). Corticosteroids and other immunosuppressive therapies are inconsistently effective in eliminating FVIII inhibitors. Accordingly, acquired FVIII inhibitors often require long-term haemostatic management.
Kobrinsky
Nathan L
NL
Roger Maris Cancer Center, North Dakota Hemophilia Treatment Center, 820 Fourth Street North, Fargo, ND 58122, USA. NathanKobrinsky@meritcare.com
Stegman
D
D
Sjolander
D
D
D000818:Animals; D000910:Antibodies, Heterophile / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood / Q000188:drug therapy / Q000276:immunology; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D019468:Disease Management; D005169:Factor VIII / Q000008:administration & dosage* / Q000276:immunology*; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000209:etiology / Q000276:immunology*; D006487:Hemostasis / Q000187:drug effects; D006801:Humans; D013552:Swine; D016896:Treatment Outcome
D000910:Antibodies, Heterophile; D001323:Autoantibodies; D003520:Cyclophosphamide; D005169:Factor VIII