variant Creutzfeldt-Jakob disease
UPDATE (FEB 2009)
Health authorities in the United Kingdom recently announced that a man with haemophilia A who died from unrelated causes was found to have evidence of infection with the agent which causes variant Creutzfeldt-Jakob disease (vCJD), the human form of "mad cow disease".
Abnormal prion proteins (the infectious agent causing vCJD) were detected in the spleen during a post mortem, which had been undertaken as part of a UK surveillance study. This is the first time that a person with haemophilia has been found to have any evidence of vCJD infection, although there has been concern that this may occur.
The patient concerned had been treated with 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 exposure to clotting factor as the most likely source of prion transmission to this patient.
It is important to note this is not the only possible source and the man had other potential risk factors. Whilst there was evidence of abnormal prion proteins, the man did not have vCJD and his death occurred due to unrelated causes. The follow-up investigations are not yet complete.
Although it can never be stated that there is a zero risk of transmission of infectious diseases by clotting factor concentrates, amongst people with haemophilia, it is those who were exposed to concentrates manufactured with UK-sourced plasma between 1980 – 2001 who are thought to be at increased risk of vCJD. Although this risk was considered theoretical and likely to be small, a number of precautions to reduce the risk were put in place and continue to apply.
Firstly from 1998, no UK-sourced plasma has been used in the manufacture of plasma products worldwide. Secondly, blood donors in Australia cannot donate blood to the Australian Red Cross Blood Service if they have resided in the UK between 1980 – 1996 for a total (cumulative) time of six months or more, or have received blood transfusions in the UK since 1 January 1980.
In Australia, recombinant treatment products which are manufactured with little or no human or animal material are used widely by people with haemophilia for their treatment in preference to plasma derived clotting factors. Nevertheless, some people with bleeding disorders still need to use plasma derivatives or choose to do so. Regulators require a number of steps to be taken in plasma derived products registered for use in Australia which result in these products being considered to be of minimal risk of vCJD. This includes donor deferral policies to exclude potentially at risk plasma, and processes and methods to reduce the risk of prions.
HFA is working with the Australian health authorities, Australian Haemophilia Centre Directors' Organisation and other organisations to keep aware of the UK situation and will provide further ongoing information to the bleeding disorders community.
People who are concerned about the implications of this information for their own health are encouraged to speak with their haemophilia doctor.
Further information about the UK situation is available from the following web sites:
UK Health Protection Agency news 17 Feb 2009
UK Haemophilia Centre Doctors' Organisation – Patient information page
UK Health Protection Agency – Variant CJD and plasma products
HFA does not give medical advice. This material is provided for general information only. Patients should refer to their treating doctor or haemophilia treatment centre for medical advice and/or advice about the treatment products they use. For contact details of haemophilia treatment centres go to Treatment Services.