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vCJD screening and its implications for transfusion--strategies for the future?
1Scottish National Blood Transfusion Service, Edinburgh & South East Scotland Blood Transfusion Centre, Royal Infirmary of Edinburgh, UK. marc.turner@snbts.csa.scot.nhs.uk
Summary
The transmission of Creutzfeldt Jakob disease (CJD) via blood products remains uncertain. While animal models show infectivity, human studies lack evidence for sporadic CJD, but variant CJD
Area of Science:
- Neurology
- Infectious Diseases
- Biochemistry
Background:
- Creutzfeldt Jakob disease (CJD) is a fatal neurodegenerative prion disease.
- The potential for CJD transmission through blood products is a significant public health concern.
- Current evidence on CJD transmission via blood transfusion in humans is inconclusive.
Purpose of the Study:
- To review the evidence regarding the transmissibility of Creutzfeldt Jakob disease (CJD) by blood products.
- To discuss the challenges and potential strategies for detecting CJD infectivity in blood.
- To evaluate the suitability of potential surrogate markers for preclinical screening of blood donations.
Main Methods:
- Review of epidemiological studies, including case-control, lookback, and surveillance data.
- Analysis of experimental data from animal models of transmissible spongiform encephalopathies.
- Assessment of diagnostic challenges due to the lack of immune response and identified nucleic acid in CJD.
- Evaluation of potential surrogate markers for CJD infectivity and disease progression.
Main Results:
- Epidemiological studies have not demonstrated sporadic CJD transmission through blood transfusion.
- Experimental models show peripheral blood infectivity in transmissible spongiform encephalopathies.
- Variant CJD may pose a different risk due to its known involvement of peripheral lymphoid tissues.
- No reliable diagnostic or surrogate markers for preclinical CJD screening in blood are currently established.
- Alpha-haemoglobin stabilizing factor is a potential surrogate marker during the incubation phase.
- Methods based on PrP(Sc) physicochemical characteristics show promise but require validation.
Conclusions:
- The risk of CJD transmission via blood transfusion remains uncertain, particularly for variant CJD.
- Development of sensitive and specific diagnostic or surrogate markers is crucial for blood safety.
- Further research is needed to validate detection methods and assess their impact on blood donor eligibility.