vCJD Abnormal Prion Protein Found in a Patient with Hemophilia at Post Mortem (February 2009)

On February 17 2009, the Health Protection Agency in the United Kingdom (UK) reported that a man with hemophilia was found at post mortem examination to have evidence of infection with the agent causing variant Creutzfeldt-Jakob disease (vCJD). The man died of unrelated causes and never showed symptoms of vCJD. As part of an ongoing study, the patient&rsquo;s spleen was biopsied after his death and evidence of the infectious agent, abnormal prion proteins, was detected. This is the first time that a person with hemophilia has been found to have any evidence of vCJD infection.<br/>
vCJD is the human disease caused by infection with an abnormal prion protein. The principal cause is the ingestion of beef infected with Bovine Spongiform Encephalopathy (BSE) or Mad Cow Disease. In the 1980s and 1990s, hundreds of thousands of cattle in the UK died from BSE. The epidemic of BSE affected Europe to a lesser extent. Fewer than 20 cattle have been diagnosed with BSE in North America. No North American has been diagnosed with vCJD from eating North American beef.<br/>
In a February 16 statement, after consulting experts in the UK, the World Federation of Hemophilia (WFH) stated,<br/>
&ldquo;This patient had severe hemophilia A, died in his early seventies, and eleven years ago he infused clotting factor concentrate which was later identified as having been contaminated with plasma from a donor who went on to develop vCJD after donation&hellip; The patient was a meat-eater, underwent surgical procedures and had received red cell transfusions, all of which are known risk factors for vCJD transmission. However, at this time, investigators believe that the most likely cause of his vCJD infection was the contaminated UK-sourced clotting factor concentrate. A complete medical history is essential for evaluating these risk factors and investigators are still working to complete that part of their study. The judgment that the infection was due to clotting factor concentrates is not final and the WFH will continue to follow this case closely, releasing more information as it becomes available.