Fast track — ArticlesPossible transmission of variant Creutzfeldt-Jakob disease by blood transfusion
Introduction
Human prion diseases include sporadic Creutzfeldt-Jakob disease (CJD), which is of unknown cause; hereditary forms associated with mutations of the prion protein gene; variant CJD (vCJD), which has been causally linked to the bovine spongiform encephalopathy (BSE) agent; and iatrogenic cases transmitted via human pituitary hormones, human dura mater grafts, corneal grafts, and neurosurgical devices. All instances of iatrogenic transmission of CJD to date have been due to crosscontamination with high-titre tissues in or adjacent to the CNS,1 and findings of epidemiological and observational studies have failed to provide evidence of transmission via blood transfusion or fractionated plasma products.2, 3 This evidence may not apply to vCJD, which is caused by a novel infectious agent for human beings and in which there is evidence of a peripheral pathogenesis different from other forms of human prion disease.4 In vCJD, prion protein is readily detectable in lymphoreticular tissues such as appendix, spleen, tonsil, and lymph nodes, whereas these tissues are negative—by comparable methods—in other forms of human prion disease.4
The possibility that vCJD might be transmitted by blood transfusion led us to start a study with the aim to identify whether vCJD was transmissible by this mechanism.
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Procedures
In 1997, a surveillance system was set up between the UK national CJD surveillance unit and the UK national blood services. Workers at the surveillance unit notified the relevant medical director of the blood services (National Blood Authority, Scottish National Blood Transfusion Service, Welsh Blood Service, Northern Ireland Blood Transfusion Service) of vCJD patients who were old enough to have donated blood (age >17 years). On receipt of this notification, workers at the blood services began
Case report
In 1996, a patient aged 62 years was transfused with 5 units of red cells at time of surgery. One of the units had been donated by a 24-year-old individual who developed symptoms of vCJD 3 years 4 months later, and who died in 2000 of pathologically confirmed vCJD.
In late 2002, 6·5 years after the blood transfusion, the recipient became withdrawn and irritable, and within 3 months, treatment with antidepressants was started— without benefit. The depression deteriorated and was associated with a
Discussion
The identification of a case of vCJD who received a blood transfusion from a donor who later died of vCJD raises the possibility that this infection was transfusion transmitted. Although statistical analysis suggests that coincidence is an unlikely explanation for this case, it is important to stress that this is a single case and there is a possibility that infection was due to dietary exposure to the BSE agent, the presumed route of zoonotic transmission of BSE.
The hypothesis of transfusion
References (19)
- et al.
Creutzfeldt-Jakob disease and blood transfusion
Lancet
(1993) - et al.
Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples
Lancet
(1999) - et al.
Transmission of BSE by blood transfusion in sheep
Lancet
(2000) - et al.
Detection of variant Creutzfeldt-Jakob disease infectivity in extraneural tissues
Lancet
(2001) - et al.
Deaths from variant Creutzfeldt-Jakob disease in the UK
Lancet
(2003) - et al.
Retrospective study of prionprotein accumulation in tonsil and appendix tissues
Lancet
(2000) - et al.
Removal of abnormal prion protein by plasma fractionation
Transfus Sci
(2000) - et al.
Iatrogenic Creutzfeldt-Jakob disease at the millennium
Neurology
(2000) - et al.
Risk of acquiring Creutzfeldt-Jakob disease from blood transfusions: systematic review of case-control studies
BMJ
(2000)
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