vCJD Abnormal Prion Protein Found In A Haemophilia Patient

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Evidence of infection with the agent (abnormal prion protein) that causes variant Creutzfeldt-Jakob Disease (vCJD) has been found at post mortem in the spleen of a person with haemophilia.

The patient, who was over 70 years old, died of a condition unrelated to vCJD and had shown no symptoms of vCJD or any other neurological condition prior to his death. The vCJD abnormal prion protein was only identified during post mortem research tests.

The Health Protection Agency is working with the UK Haemophilia Centre Doctors Organisation to ensure all patients with bleeding disorders are made aware of this preliminary information which is being further investigated. This new finding will not change the way patients with haemophilia are cared for or treated.

A final view as to how vCJD abnormal prion protein was transmitted to this haemophilia patient has yet to be reached because investigations are continuing to determine the most likely route of transmission. It is known that the patient had been treated with several batches of UK sourced clotting factors before 1999, which is when measures to improve the safety of blood in relation to vCJD were introduced. The patient's treatment had included one batch of Factor VIII that was manufactured using plasma from a donor who went on to develop symptoms of vCJD six months after donating the plasma in 1996.

This is the first time that vCJD abnormal prion protein has been found in a patient with haemophilia, or any patient treated with plasma products. This new finding, however, does not change the public health vCJD 'at risk' status of patients with bleeding disorders.

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Haemophilia patients have previously been informed by their doctors of their possible increased risk of exposure to vCJD via clotting factors. In 2004 all patients with bleeding disorders who had been treated with UK-sourced pooled plasma products between 1980 and 2001 were told that, owing to potential vCJD infectivity from these products they were to be classified as at-risk of vCJD for public health purposes.

Professor Mike Catchpole, Director of the Health Protection Agency's Centre for Infections, said:

"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. 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.

The priority is to ensure that patients are informed of this development and have access to the latest information and specialist advice from their own haemophilia centre doctor as soon as possible.

“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.

“Since the risk of vCJD transmission through blood was first considered, a number of precautionary measures have been introduced to minimise the risk from the UK blood supply. 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.”

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