Sporadic Creutzfeldt-Jakob Disease in 2 Plasma Product Recipients, United Kingdom

Emerging Infectious Diseases
Patrick UrwinR G Will

Abstract

Sporadic Creutzfeldt-Jakob disease (sCJD) has not been previously reported in patients with clotting disorders treated with fractionated plasma products. We report 2 cases of sCJD identified in the United Kingdom in patients with a history of extended treatment for clotting disorders; 1 patient had hemophilia B and the other von Willebrand disease. Both patients had been informed previously that they were at increased risk for variant CJD because of past treatment with fractionated plasma products sourced in the United Kingdom. However, both cases had clinical and investigative features suggestive of sCJD. This diagnosis was confirmed in both cases on neuropathologic and biochemical analysis of the brain. A causal link between the treatment with plasma products and the development of sCJD has not been established, and the occurrence of these cases may simply reflect a chance event in the context of systematic surveillance for CJD in large populations.

References

Jun 14, 1991·Science·S B Prusiner
Nov 7, 2003·The New England Journal of Medicine·Markus GlatzelAdriano Aguzzi
Jan 1, 2014·Emerging Infectious Diseases·Jean Yves DouetOlivier Andreoletti
Jan 23, 2016·Science Translational Medicine·Eric Vallabh MinikelDaniel G MacArthur

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Citations

Jan 29, 2019·Transfusion Medicine·C ReynoldsS R Brailsford
Feb 21, 2018·The Journal of General Virology·Cyrus BettLuisa Gregori
May 21, 2020·Frontiers in Immunology·Odette Leiter, Tara L Walker
May 12, 2021·Nature Reviews. Neurology·Neil WatsonSuvankar Pal

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