title: The successful removal of a bleeding intracranial tumour in a severe haemophiliac using an adjusted dose continuous infusion of monoclonal factor VIII.
abstract: Ten per cent of patients with haemophilia A develop intracranial haemorrhage (ICH) with a mortality rate of 30% and an incidence of psycho-neurological sequelae in 50% of survivors. ICH may be spontaneous or in association with trauma and other pathology. The generally recommended management is conservative replacement therapy using bolus injections of factor VIII and no neurosurgical intervention. Adjusted dose continuous infusion therapy provides an alternative method of factor VIII administration that is simple, more cost effective and safer through the maintenance of stable plasma VIII:C levels. This method has been successfully used to cover general surgery and the conservative treatment of subarachnoid haemorrhage but is not widely used due to unfamiliarity with the technique. This paper describes the use of continuous infusion of factor VIII concentrates to cover the successful neurosurgical management of a young man with severe haemophilia A who presented with an ICH associated with a bleeding choroid plexus tumour. Surgery was complicated by the development of a factor VIII inhibitor which disappeared following treatment with an immune-tolerance induction programme.
authors.affiliation: Haemophilia Reference Centre, St Thomas's Hospital, London, UK.
authors.forename: H A
authors.initials: HA
authors.lastname: Doughty
authors.forename: J
authors.initials: J
authors.lastname: Coles
authors.forename: K
authors.initials: K
authors.lastname: Parmar
authors.forename: P
authors.initials: P
authors.lastname: Bullock
authors.forename: G F
authors.initials: GF
authors.lastname: Savidge
mesh_terms: D000293:Adolescent; D002543:Cerebral Hemorrhage / Q000188:drug therapy* / Q000209:etiology; D016545:Choroid Plexus Neoplasms / Q000150:complications / Q000601:surgery*; D003399:Craniotomy; D005169:Factor VIII / Q000008:administration & dosage / Q000493:pharmacokinetics / Q000627:therapeutic use*; D006406:Hematoma / Q000188:drug therapy / Q000209:etiology; D006429:Hemiplegia / Q000209:etiology; D006801:Humans; D007260:Infusion Pumps; D007262:Infusions, Intravenous; D008297:Male; D010212:Papilloma / Q000150:complications / Q000601:surgery*
chemical_list: D005169:Factor VIII