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Risk of Transmission of Creutzfeldt–Jakob Disease by Blood Transfusion

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Abstract

Early epidemiological studies on sporadic Creutzfeldt–Jakob disease did not identify blood transfusion as a risk factor for the disease. However, the emergence of variant Creutzfeldt–Jakob disease (vCJD) in 1996 and the identification of PrPSc in lymphoid tissues in this novel disorder led to concerns that transmission of infectivity by blood transfusion might be a possibility. These concerns were fully realised in 2004, when the first case of vCJD associated with transmission by blood transfusion was identified in a recipient who was a methionine homozygote at codon 129 in the prion protein gene, as in all other vCJD patients. Other similar cases have subsequently emerged, along with cases of asymptomatic vCJD infection in a blood transfusion recipient and a plasma product recipient, both of whom were heterozygous at codon 129 of the prion protein gene. This chapter reviews the experimental evidence for the transmission of prion infectivity by blood transfusion in a range of experimental models, discusses the evidence for the transmission of vCJD by blood transfusion and plasma products and considers the future possibilities for the development and potential uses of blood-based screening tests for human prion diseases.

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Acknowledgements

The work of the National CJD Research & Surveillance Unit is supported by the Department of Health, UK, and the Scottish Government. Dr. Alexander Peden has been supported by grants funding from the Scottish Government Health Directorate Chief Scientists Office. The views expressed in the publication are those of the authors and not necessarily those of the Department of Health, UK. The authors would like to thank relatives of patients for the opportunity to conduct research on tissue specimens at the National CJD Research & Surveillance Unit that contributed to the identification of blood transfusion-related transmission of variant CJD.

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  1. National Creutzfeldt-Jakob Disease Research & Surveillance Unit, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Edinburgh, EH4 2XU, UK

    Alexander H. Peden Ph.D., Mark W. Head Ph.D. & James W. Ironside F.R.C.Path.

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  1. Alexander H. Peden Ph.D.
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  2. Mark W. Head Ph.D.
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Correspondence to James W. Ironside F.R.C.Path. .

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  1. , Department of Pathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Wen-Quan Zou

  2. , Department of Neuropathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Pierluigi Gambetti

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Peden, A.H., Head, M.W., Ironside, J.W. (2013). Risk of Transmission of Creutzfeldt–Jakob Disease by Blood Transfusion. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5338-3_8

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