Haemophilia patient had variant CJD agent in spleenBMJ 2009; 338 doi: https://doi.org/10.1136/bmj.b705 (Published 18 February 2009) Cite this as: BMJ 2009;338:b705
A postmortem of a 70 year old patient with haemophilia has shown that he had the abnormal prion protein for Creutzfeldt-Jakob Disease (vCJD) in his spleen. It is the first time the vCJD agent has been found in a patient with haemophilia or anyone treated with a plasma product.
The patient’s death was unrelated to vCJD, and he had no symptoms of the disease or any other neurological condition before his death. However, the Health Protection Agency is working with the UK Haemophilia Centre Doctors’ Organisation to alert people with haemophilia to the postmortem results.
Although it has not been confirmed how transmission occurred, the patient had received one batch of factor VIII that was manufactured using plasma from a donor who developed symptoms of vCJD six months after donating the plasma in 1996. This was one of several batches of United Kingdom sourced clotting factors the patient received before 1999, when measures to improve the safety of blood in relation to vCJD were introduced.
A spokesperson from the Health Protection Agency said it would not alter the agency’s advice to people with haemophilia.
In 2004, all patients with bleeding disorders who had been treated with UK sourced pooled plasma products between 1980 and 2001 were told that they were at risk of vCJD and were therefore excluded from donating organs.
“This new finding may indicate that what was until now a theoretical risk may be an actual risk to certain individuals who have received blood plasma products, although the risk could still be quite low,” said Professor Mike Catchpole, director of the Health Protection Agency’s centre for infections. “We recognise that this finding will be of concern for persons with haemophilia who will be awaiting the completion of the ongoing investigations and their interpretation.
“This finding does not change our understanding of the risk from vCJD for other people in any specific way. But it does reinforce the importance of the precautionary measures that have been taken over the years.”
UK plasma has not been used for the manufacture of clotting factors since 1999 and synthetic clotting factors are provided for all patients for whom they are suitable, he said.
Cite this as: BMJ 2009;338:b705
For further information see www.hpa.org.uk.