Elsevier

The Lancet

Volume 363, Issue 9407, 7 February 2004, Pages 417-421
The Lancet

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Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion

https://doi.org/10.1016/S0140-6736(04)15486-XGet rights and content

Summary

Background

Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease caused by infection with the agent of bovine spongiform encephalopathy (BSE). Epidemiological evidence does not suggest that sporadic CJD is transmitted from person to person via blood transfusion, but this evidence may not apply to vCJD. We aimed to identify whether vCJD is transmissible through blood transfusion.

Methods

The national CJD surveillance unit reported all cases of probable or definite vCJD to the UK blood services, which searched for donation records at blood centres and hospitals. Information on named recipients and donors was provided to the surveillance unit to establish if any matches existed between recipients or donors and the database of cases of vCJD. Recipients were also flagged at the UK Office of National Statistics to establish date and cause of death.

Findings

48 individuals were identified as having received a labile blood component from a total of 15 donors who later became vCJD cases and appeared on the surveillance unit's register. One of these recipients was identified as developing symptoms of vCJD 6·5 years after receiving a transfusion of red cells donated by an individual 3·5 years before the donor developed symptoms of vCJD.

Interpretation

Our findings raise the possibility that this infection was transfusion transmitted. Infection in the recipient could have been due to past dietary exposure to the BSE agent. However, the age of the patient was well beyond that of most vCJD cases, and the chance of observing a case of vCJD in a recipient in the absence of transfusion transmitted infection is about 1 in 15 000 to 1 in 30 000.

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.

Section snippets

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)

There are more references available in the full text version of this article.

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