Attributable testing for abnormal prion protein, database linkage, and blood-borne vCJD risks.

Abstract

CONTEXT: National prospective collection of tonsillar tissue to be tested anonymously for abnormal lymphoreticular accumulation of prion protein (PrP) was approved to begin in the UK in 2004. The UK is not, however, testing autopsy specimens attributably for abnormal PrP (PrP(SC)) so that recipients at risk after a blood transfusion from, or exposed to surgical instruments from, a deceased carrier of variant Creutzfeldt-Jakob disease (vCJD) can be followed up to quantify transmission risks. In Switzerland, surveillance for subclinical vCJD includes unconsented testing in autopsies: consented testing of tonsillar tissue is potentially attributable to interrupt human-to-human vCJD transmission or treat it. STARTING POINT: The UK announced its first case of probable blood-borne vCJD transmission in December, 2003, and first detected a case of probable blood-borne subclinical vCJD in July, 2004. To reduce the possible risk of onward transmission to other people, UK patients who had received vCJD-implicated plasma products are being contacted. They, and their general practitioner, are asked to inform anyone giving them medical, surgical, or dental treatment, and the patients must refrain from donating blood, tissues, or organs. WHERE NEXT? Prudent additional surveillance options for human PrP(SC)--particularly at autopsy or to sanction the release of quarantined operation sets pending effective decontamination--can be costed by reference to results for cattle and sheep. Some ethical or legal impediments to the UK's potentially-attributable testing for PrP(SC) may yet be rued.