Creutzfeldt-Jacob Disease Question Asked by Lord Walton of Detchant P-Capt filter
To ask Her Majesty's Government what is their estimate of the number of individuals in the United Kingdom carrying the prion responsible for the non-variant Creutzfeldt-Jacob Disease (CJD); and whether they propose to recommend the widespread use of the P-CAPT filter on blood available for transfusion, so as to prevent the transmission of this infective agent.[HL14081]
The Parliamentary Under-Secretary of State, Department of Health (Earl Howe): Human prion diseases not classified as variant (v) Creutzfeldt-Jakob disease (CJD) include sporadic CJD, iatrogenic CJD and familial CJD (including Gerstmann-Straussler-Scheinker (GSS) syndrome). While there are no reliable data on the prevalence of these conditions, in total they affect about 1:1,000,000 of the population per
15 Dec 2011 : Column WA284
annum. Data on deaths due to all forms of CJD are published monthly by the United Kingdom Creutzfeldt-Jakob disease Research and Surveillance Unit and can be found at: www.cjd.ed.ac.uk/figures.htm.
The P-Capt filter is marketed by Macopharma for the removal of prions from leucodepleted blood to reduce the risk of transmission of vCJD. While there is evidence of presumed transmissions of vCJD via blood transfusions from donors who later went on to develop clinical vCJD, there is no evidence of other forms of CJD being transmitted by transfusion.
The potential use of the P-Capt filter, to reduce the risk of potential transmission of vCJD, is under consideration by the Advisory Committee on the Safety of Blood Tissues and Organs (SaBTO) and will be on the agenda for the SaBTO's first 2012 meeting to be held on 9 March 2012.
Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD, and GSS Blood Specimens: the Baxter Study
Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3 1Fondation Alliance BioSecure, France; 2University of South Alabama, USA; 3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK; 5Baxter BioSience, Austria
Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy (TSE) have documented blood infectivity in both the pre-clinical and clinical phases of disease. Results in a (presumably more appropriate) non-human primate model have not been reported.
Objective: To determine if blood components (red cells, white cells, platelets, and plasma) from various forms of human TSE are infectious.
Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease (vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from chimpanzees infected with either sCJD or Gerstmann-StrÃ¤ussler-Scheinker disease (GSS). Animals were monitored for a period of 5 years, and all dying or sacrificed animals had post-mortem neuropathological examinations and Western blots to determine the presence or absence of the misfolded prion protein (PrPTSE).
Results: No transmissions occurred in any of the animals inoculated with blood components from patients with sporadic or variant CJD. All donor chimpanzees (sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase plasmapheresis, several months earlier than the expected onset of illness. One monkey inoculated with purified leukocytes from a pre-clinical GSS chimpanzee developed disease after 36 months.
Conclusion: No infectivity was found in small volumes of blood components from 4 patients with sporadic CJD and 3 patients with variant CJD. ***However, a single transmission from a chimpanzee-passaged strain of GSS shows that infectivity may be present in leukocytes, and the shock of general anaesthesia and plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.
Saturday, September 5, 2009
TSEAC MEETING FEBRUARY 12, 2004 THE BAXTER STUDY GSS
Wednesday, June 29, 2011
TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products
Wednesday, August 24, 2011
All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD
Wednesday, August 24, 2011
There Is No Safe Dose of Prions
stupid is, as stupid does, and some times, you just can’t fix stupid $$$