He had received over 9000 units of FVIII prepared from plasma pools that included donations from a presumed variant CJD-infected UK donor. He had also received 14 units of red blood cells and had undergone several surgical and invasive endoscopic procedures. Estimates of the relative levels of risk to which this individual was exposed through diet, surgery/endoscopy, blood transfusion and receipt of
plasma products suggested that by far the most likely route through which this individual was infected was the receipt of contaminated plasma products [19]. This case represents the first demonstration of variant CJD infection in a haemophilic patient; no clinical cases of variant CJD have been identified to date in a patient treated selleck chemicals with UK-derived clotting factor concentrates. Many of the concerns over the risks of transmission of variant CJD by blood and plasma components could be reduced if a screening test was available
to detect asymptomatic infection. The analytical and practical challenges of developing such a test are considerable, but a number of different approaches are currently underway [26,37]. One of these has been reported recently to be able to detect variant CJD prions in the blood of non-human primates experimentally infected with BSE [38]. Confirmation of this encouraging claim, as well as an assessment of the test analytical parameters, in a peer reviewed publication is awaited. In the meantime, pressure to implement such Palbociclib ic50 a test in individuals deemed to be at increased risk of variant CJD is likely to increase, particularly in the UK. However, even if a screening test
for variant CJD was to become available soon, additional concerns have been voiced over the required sensitivity and specificity, particularly in the absence of a confirmatory test [26]. The need for informed consent for such tests is currently under debate; the lack of any prophylaxis or treatment for prion diseases raises questions over any potential benefit for an individual undergoing testing. Continuing surveillance for variant CJD is required to assess more fully the risks to patients with bleeding disorders who have been exposed see more to potentially infected plasma products. The National Creutzfeldt–Jakob Disease Surveillance Unit (NCJDSU) is supported by the Department of Health and the Scottish Executive. The Brain Bank in NCJDSU is supported by the Medical Research Council (G0900580). I am grateful to Ms Diane Ritchie for providing Fig. 1, to Dr Mark Head for providing Fig. 2 and to the NCJDSU Biomedical Scientists for expert support. I am also indebted to the relatives of patients with prion diseases for giving consent for the use of tissues in research. The author stated that he had no interests which might be perceived as posing a conflict or bias.