THE SUCCESSFUL REMOVAL OF A BLEEDING INTRACRANIAL TUMOR IN A SEVERE HEMOPHILIAC USING AN ADJUSTED-DOSE CONTINUOUS-INFUSION OF MONOCLONAL FACTOR-VIII

Ten per cent of patients with haemophilia A develop intracranial haemo rrhage (ICH) with a mortality rate of 30% and an incidence of psyche-n eurological sequelae in 50% of survivors. ICH may be spontaneous or in association with trauma and other pathology. The generally recommende d management is conservative replacement therapy using bolus injection s of factor VIII and no neurosurgical intervention. Adjusted dose cont inuous infusion therapy provides an alternative method of factor VIII administration that is simple, more cost effective and safer through t he maintenance of stable plasma VIII:C levels. This method has been su ccessfully used to cover general surgery and the conservative treatmen t of subarachnoid haemorrhage but is not widely used due to unfamiliar ity with the technique. This paper describes the use of continuous inf usion of factor VIII concentrates to cover the successful neurosurgica l management of a young man with severe haemophilia A who presented wi th an ICH associated with a bleeding choroid plexus tumour, Surgery wa s complicated by the development of a factor VIII inhibitor which disa ppeared following treatment with an immune-tolerance induction program me.