A new case of probable variant Creutzfeldt-Jakob disease
(vCJD) has recently been diagnosed in a patient in the United Kingdom (UK)
who received a blood transfusion from a donor who later developed vCJD [1].
This patient, who is still alive and is under the care of doctors at the National
Prion Clinic, is the third case of vCJD infection in the UK associated with
transfusion [2].
The first case of vCJD disease associated with blood transfusion was identified
in December 2003 [3]. This patient developed vCJD six and a half years after
receiving a transfusion of red blood cells donated by an individual who
developed symptoms of vCJD three and a half years after donation [4].
Another case of vCJD 'infection' was identified a few months later in a
recipient of red blood cells from a donor who developed symptoms of vCJD
18 months after the donation [5]. This second patient died from causes unrelated
to vCJD five years after transfusion. Postmortem 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 [6].
The most recent patient developed vCJD almost eight years after receiving
a transfusion of red blood cells from a donor who developed vCJD about 20
months after donating this blood [1]. Each of the three infected recipients
received blood from different donors.
To date, 160 cases of vCJD have been identified in the UK. A collaborative
study (the Transfusion Medicine Epidemiology Review) between the National
Blood Services, the National CJD Surveillance Unit and the Office for National
Statistics has been conducted since 1997 to collect evidence about transmission
of CJD or vCJD via the blood supply [7]. Review of data at blood centres has
found records for 23 of the 160 vCJD cases (prior to their vCJD diagnosis).
For 18 of these 23 cases, blood components were issued to hospitals for transfusion,
and 66 recipients of these vCJD-implicated blood donations have been identified.*
Forty of these 66 recipients have died, including the two previous patients
with probable transfusion-associated vCJD [4,6]. The small group of living
recipients of vCJD-implicated blood transfusion have been informed of their
potential exposure to vCJD by blood transfusion. Some were contacted in late
2003 and early 2004, and some in 2005. They were asked to take certain precautions
to reduce the risk of onward person-to-person transmission of vCJD during
medical procedures.
All three infected recipients identified to date received non-leucodepleted
red blood cells. 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 infected donor is uncertain.
The risk of vCJD infectivity in blood has also resulted in certain other
groups of individuals being categorised as ‘at risk of vCJD for public health
purposes’. These groups have been informed and asked to take public health
precautions. The groups include certain recipients of plasma products [8],
individuals who have donated blood to patients who developed vCJD [9] and
certain recipients of blood from donors to patients who developed vCJD [10].
To date, there have been no vCJD cases associated with receipt of plasma
products, or in these other groups of individuals that have been categorised
as ‘at risk’.
This third case of vCJD infection associated with blood transfusion provides
further evidence that vCJD can be transmitted 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 [11].
Numbers of vCJD cases countries other than the UK remain small: by January
2006, there had been 15 cases reported in France, 4 in Ireland, 2 in the
United States, and 1 each in Canada, Italy, Japan, the Netherlands, Portugal,
Saudi Arabia and Spain [12].
*Correction. There was an error in
this article as originally published on 9 February. The original text from
the beginning of the paragraph until the asterisk read 'To date, 160 cases
of vCJD have been identified in the UK. Of these, 23 are known to have donated
blood before the diagnosis of vCJD. A collaborative study (the Transfusion
Medicine Epidemiology Review) between the National Blood Services, the National
CJD Surveillance Unit and the Office for National Statistics has been conducted
since 1997 to collect evidence about transmission of CJD or vCJD via the blood
supply [7]. Blood donations have been traced for 18 of the 23 known donors,
and 66 recipients of these vCJD-implicated blood donations have been identified.'
This was an incorrect description of the study results,
and the mistake was corrected in both Eurosurveillance, and the CDR
Weekly article (reference 2) on 16 February 2006.
Eurosurveillance Editorial Office
This article has been adapted from reference 2