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Risk of Transmission of Creutzfeldt–Jakob Disease by Blood Transfusion

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Prions and Diseases

Abstract

Early epidemiological studies on sporadic Creutzfeldt–Jakob disease (CJD) did not identify blood transfusion as a risk factor for the disease. However, the emergence of variant CJD (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 at that point in time. 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

This report is based on independent research commissioned and funded by the Policy Research Programme, Department of Health and Social Care and the Scottish Government [The National CJD Research and Surveillance Unit (NCJDRSU), PR-ST-0614-00008_18]. The views expressed in this publication are those of the author(s) and not necessarily those of the NHS, the NIHR, the Department of Health and Social Care, the Scottish Government, “arms” length bodies or other government departments. The authors wish to thank Emeritus Professor James Ironside and Dr Mark W Head for their work in the preparation of the previous version of this book chapter. The authors would like to thank relatives of patients for the opportunity to conduct research on tissue specimens at the National CJD Research and Surveillance Unit that contributed to the identification of blood transfusion-related transmission of variant Creutzfeldt–Jakob disease.

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

    Alexander H. Peden & Marcelo A. Barria

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  1. Alexander H. Peden
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Correspondence to Alexander H. Peden .

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  1. Departments of Pathology and Neurology, National Prion Disease Pathology Surveillance Center, National Center for Regenerative Medicine Case Western Reserve University, School of Medicine, Cleveland, OH, USA

    Wen-Quan Zou

  2. Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Pierluigi Gambetti

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Peden, A.H., Barria, M.A. (2023). Risk of Transmission of Creutzfeldt–Jakob Disease by Blood Transfusion. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-20565-1_12

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