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Medical Advisory #409: Signs of Variant Creutzfeldt-Jakob Disease Found in a UK Patient with Hemophilia; No Added Risk Seen for U.S. Patients

February 18, 2009

Health authorities in the United Kingdom have announced that a man with hemophilia A who died of unrelated causes was found at post-mortem examination to have evidence of infection with the agent causing variant Creutzfeldt-Jakob disease (vCJD), the human form of “mad cow disease.” The discovery was made when a biopsy was performed on the patient’s spleen as part of an ongoing UK surveillance study, and signs of abnormal prion proteins, the infectious agent causing vCJD, were detected. This is the first time that a person with hemophilia has been found to have any evidence of vCJD infection.

Eleven years ago, this patient infused UK-produced clotting factor concentrate, which was later identified as having been made from plasma from a donor who developed vCJD after making the donation. Health authorities in the UK have identified this clotting factor exposure as the likely source of prion transmission to this patient, although it is not the only possible source, and the follow-up clinical research is not yet complete.

It is important to note that only individuals exposed to UK-sourced plasma infused between 1980 and 2001 are considered to be at elevated risk for vCJD compared to the general population. These products were used primarily by patients in the UK, although some products were exported to Brazil, Brunei, India, Jordan, Oman, Singapore, Turkey, and United Arab Emirates. A few individuals in the US used a UK-produced FXI product during this time, but no vCJD-implicated plasma was used in its production.

Currently, UK-sourced plasma is not used in any product worldwide, and it has never been used in products licensed for use in the United States. In the US, plasma donors who have spent time in the UK are deferred from donating blood or plasma. Therefore, there should be no change in the safety profile of US-licensed products or in any treatment recommendations.

The Medical and Scientific Advisory Council (MASAC) and the Blood Safety Working Group of NHF are working closely with relevant U.S. and international agencies to monitor this issue and distribute any new information that becomes available. Additional information about this case is available from these UK Web sites:

Information about vCJD and other blood and product safety issues is also available on the 
NHF Web site, and on the Web site of the World Federation of Hemophilia (WFH).

Patients with individual concerns should contact their treatment center physician.

PHYSICIANS: Please distribute this information to all providers in your area who treat patients with hemophilia.

CHAPTERS: Please distribute this information to your membership.

Please sign up for the Patient Notification System (PNS) to be notified directly about the latest recall or withdrawal of recombinant and plasma products. The system is confidential and time sensitive. It is administered by an independent third-party organization and is free of charge.  To enroll in the PNS online, please go to:

This material is provided for your general information only.  NHF does not give medical advice or engage in the practice of medicine.  NHF under no circumstances recommends treatment for specific individuals and in all cases recommend that you consult your physician or local hemophilia treatment center before pursuing any course of treatment.