Researchers report the details of the UK’s third case of variant Creutzfeldt-Jakob disease (vCJD) associated with blood transfusion in an Article in this week’s issue of The Lancet. The case highlights the risk faced by other recipients of vCJD infected blood and suggests that blood transfusion is an efficient route of transmission for vCJD.
In 2004 two transfusion-associated cases of vCJD prion infection were reported. The patients both had a single blood transfusion from donors who later developed vCJD. One of these patients developed clinical signs of vCJD 6.5 years post-transfusion and died 13 months later. The second patient did not develop vCJD and died of an unrelated cause 5 years post-transfusion. The diagnosis of the third case of transfusion-related vCJD was announced in February this year.
The young male patient developed symptoms of vCJD about 6 years after receiving a blood transfusion from a donor who developed symptoms of vCJD 20 months after donating blood. Progressive symptoms. including impaired concentration and poor balance. led to his referral to the NHS National Prion Clinic at the National Hospital for Neurology and Neurosurgery, London. The patient decided to enroll into a trial testing a treatment called quinacrine. He received 300mg of quinacrine daily on the trial. However, he continued to decline and eventually became bed-bound. The patient died 8 years and 8 months after the vCJD implicated transfusion. Autopsy confirmed the vCJD diagnosis and showed prion infection.
There are 24 living individuals who are known to have received a blood transfusion in the UK from a donor who later developed vCJD. All these individuals have now been informed of their at risk status. All should be given the opportunity of immediate access to specialist advice, assessment and long-term support, write John Collinge (National Prion Clinic and MRC Prion Unit, National Hospital for Neurology and Neurosurgery, London, UK) and colleagues in their Article.
They add: "This case illustrates the importance of diagnosis as early as possible in the clinical course. The patient was able to consider the option of joining a therapeutic clinical trial, to fully discuss the implications of his diagnosis, and to communicate his wishes to the clinical team and his family, making decisions and arrangements about his future care and other affairs. Specialist monitoring and investigation of high-risk groups, such as recipients of blood transfusion from a donor who developed vCJD, should allow the condition to be recognised at an early stage."
In an accompanying Comment Kumanan Wilson (Toronto General Hospital, Toronto, Ontario, Canada) and Maura Ricketts (Public Health Agency of Canada) state: "This third case considerably strengthens the inference that transfusion transmission is possible, and suggests that the causative prion can be efficiently transmitted via this route."
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