Post mortem finding of asymptomatic variant Creutzfeldt - Jakob Disease (vCJD)

The abnormal prion protein was found in a UK resident with haemophilia.

Issued 15 April 2009

A person with haemophilia has been found to have evidence of infection with the agent (abnormal prion protein) that causes variant Creutzfeldt-Jakob Disease (vCJD), only in his spleen, at post mortem. 

This haemophilia patient had been treated in the 1990s with several batches of UK-sourced clotting factors, including one batch of factor VIII that was manufactured using plasma from a donor who went on to develop vCJD.  The haemophilia patient was in his 70s when he died of a condition unrelated to vCJD.  He had no signs or symptoms of vCJD or other neurological disease when alive.

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.   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.

All Haemophilia Centres have been contacting their patients throughout the UK to give them information about this new finding.

Patients who have a bleeding disorder or congenital antithrombin III deficiency and who received clotting factors or antithrombin derived from UK-sourced plasma between 1980 and 2001, were told in 2004 that they have an increased risk of vCJD, and should follow public health advice. Other patients with bleeding disorders who do not have an increased risk of vCJD, do not need to take any action.

Such patients were to be informed that they were ‘at risk of vCJD for public health purposes’, and that their ‘at risk’ status would be recorded in their hospital medical records and primary care notes.  Patients who had NOT received UK-sourced pooled factor concentrate or antithrombin between 1980 and 2001 had this fact clearly recorded.

Public health advice given to ‘at risk’ patients in 2004 stated that they should not donate blood, organs or tissues (many patients who have received plasma products may already be excluded from donation because of their underlying condition).

Further information is available on the Health Protection Agency (HPA) website.

This information has been provided by the HPA.  The MHRA (in their role as Competent Authority for blood) has notified the European Commission, who have circulated an alert to all European blood and tissue and cells Competent Authorities.

Actions for establishments

Establishments should ensure that staff responsible for donor selection and evaluation are informed of this recent finding and are aware of the public health advice given above.  In particular, establishments are advised that:

• the medical records of any patients with bleeding disorders are checked for confirmation of their ‘at risk’ status.
• patients designated ‘at risk of vCJD for public health purposes’ should not donate blood, organs or tissues.

Ongoing advice and guidance

All human application establishments are advised to review their selection criteria for donors of tissue and/or cells in light of this information.

Establishments are reminded that under the Guide to Quality and Safety Assurance of Human Tissues and Cells for Patient Treatments Annex A:

“Donors must be excluded from donation if any of the following criteria applies:

1.1.4 Risk of transmission of diseases caused by prions.  This risk applies, for example to:

a) people diagnosed with Creutzfeldt-Jakob disease, or variant Creutzfeldt-Jakob disease, or having a family history of non-iatrogenic Creutzfeldt-Jakob disease;

b) people with a history of rapid progressive dementia or degenerative neurological disease, including those of unknown origin;

c) recipients of hormones derived from the human pituitary gland (such as growth hormones) and recipients of grafts of cornea, sclera and dura mater, and persons that have undergone undocumented neurosurgery (where dura mater may have been used).

"For variant Creutzfeldt-Jakob disease, further precautionary measures may be recommended.”

Examples of further precautionary measures which may be considered based on an analysis of the risks related to the application of the specific tissue / cells include:

• exclusion of donors who have received allogeneic human tissue or organs transplanted since 1 January 1980
• exclusion of donors who have received a transfusion of blood components (including plasma exchange), blood products or their derivatives since 1 January 1980
• exclusion of donors who have received immunoglobulin therapy since 1 January 1980.

Please contact Victoria Gauden on 020 7211 3409 or email victoria.gauden@hta.gov.uk if you would like any further advice on any of the above points or you are storing tissue which you think may be affected.

Further guidance in relation to your obligations under the European Union Tissues and Cells Directives as implemented by the Human Tissue (Quality and Safety for Human Application) Regulations 2007 can be found by referring to the Guide to Quality and Safety Assurance of Human Tissues and Cells for Patient Treatments as implemented by HTA Directions 003/2010.