Retrospective neuropathological review of prion disease in UK haemophilic patients

In 1996, the CJD surveillance unit in Edinburgh, UK described nvCJD which w as thought to be the human equivalent of bovine spongiform encephalopathy ( BSE). The identification of prion protein in the tonsil of an affected indi vidual has raised the question of transmission of nvCJD via blood products. This study examines the post mortem brains of 33 patients who were treated with clotting factor concentrate of predominately UK donor source during t he years 1962-1995. The brains were examined by conventional histological m ethods and also for the prion protein using monoclonal antibodies KG9 and 3 F4. No evidence of spongiform encephalopathy was found and the immunocytoch emistry was negative for PrP in all cases. It is concluded that, at present , there is no evidence for the transmission of nvCJD via clotting factor co ncentrate to patients with haemophilia.