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Published: 19 January 2007, Volume 1, No 3 (PDF file, 583 KB)

Volume 1 No 3; 19 January 2007

 

Fourth case of transfusion-associated variant-CJD infection

A new case of probable variant Creutzfeldt-Jakob disease (vCJD) has recently been diagnosed in a patient who received a blood transfusion from a donor who later developed vCJD [1]. This is the fourth case of probable transfusion transmission of vCJD infection in the United Kingdom (UK). Three of the four recipients developed symptoms of vCJD.

The first symptomatic case of vCJD disease associated with blood transfusion was identified in December 2003. This individual developed vCJD six and a half years after transfusion of red cells donated by an individual who developed symptoms of vCJD three and a half years after donation.

A second case of vCJD 'infection' was identified a few months later in a recipient of red cells from a donor who developed symptoms of vCJD 18 months after donation. This case died from causes unrelated to vCJD five years after transfusion. Post-mortem investigations found abnormal prion protein in the spleen and a cervical lymph node, but not in the brain, and no pathological features of vCJD were found.

A third case developed symptoms of vCJD 6 years and died eight years and eight months after receiving a transfusion of red blood cells from a donor who developed vCJD about 20 months after this blood was donated. These three cases have been published as case reports [2-4] and in findings of the ongoing collaborative study between the National Blood Services, the National CJD Surveillance Unit, and the Office for National Statistics to collect evidence about transmission of CJD or vCJD via the blood supply [5].

The new, and fourth, case of infection developed symptoms of vCJD eight and a half years after receiving a transfusion of red blood cells from a donor who developed vCJD about 17 months after this blood was donated [1]. The donor to this case also donated the vCJD-implicated blood transfused to the third case. As for all other reported clinical vCJD cases that have been tested for genotype, this patient is a methionine homozygote at codon 129 of the prion protein gene. The patient is still alive.

All four cases had received transfusions of non-leucodepleted red blood cells between 1996 and 1999. Since October 1999, leucocytes have been removed from all blood used for transfusion in the UK. The effect of leucodepletion on the reduction of the risk of transmission of vCJD from an infective donation is uncertain.

This fourth case of vCJD infection associated with blood transfusion further increases the level of concern about the risk of vCJD transmission between humans by blood transfusion, although much remains unknown. This reinforces the importance of the existing precautions that have been introduced to reduce the risk of transmission of vCJD infection by blood and blood products [6]. No cases of vCJD have been associated with fractionated plasma products. The small group of living recipients of vCJD-implicated blood transfusion in the UK have been informed of their potential exposure to vCJD by blood transfusion, asked to take certain precautions to reduce the risk of onward person-to-person transmission of vCJD during healthcare, and offered specialist neurological evaluation and advice.

References

1. Health Protection Agency. Fourth case of variant CJD associated with blood transfusion (Press Release). London: HPA, 18 January 2007. Available at
<http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm>.

2. Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, et al. Possible transmission of variant CJD disease by blood transfusion. Lancet 2004; 363;417-21.

3. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004; 364;527-9.

4. Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, et al. Clinical presentation and pre-mortem diagnosis of blood transfusion-associated variant CJD. Lancet 2006; 368: 2061-7.

5. Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiology review study. Vox Sang 2006; 91(3): 221-30.

6. Department of Health. Further precautions to protect blood supply Press release 2004/0104. London: Department of Health, 16 March 2004. Available at
<http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?
CONTENT_ID=4076608&chk=MTwE%2Bl
>.