Prion Surveillance in Primary Immunodeficiency Patients

Chief Investigator: Professor Richard Knight

Variant Creutzfeldt-Jakob Disease (vCJD) is a very rare disease, associated with an abnormal form of a naturally occurring protein (the prion protein), the presence of which can be detected in certain body tissues. The Primary Immunodeficiency Study aims to find out whether any evidence of this abnormal prion protein can be found in antibody deficient patients who received certain UK-sourced immunoglobulin products between 1996 and 2000. The products were made from plasma from UK donors and patients treated with these products may have been exposed to vCJD.

The study involves immunology teams and patients throughout the UK. Participants are followed over several years, testing any available tissue (for example, the tissue left over from routine biopsies) and blood (when a suitable test becomes available) for the abnormal prion protein that causes prion disease. Participants can also agree to donate tissues obtained from a post-mortem, as this is the best way to collect samples that help us confidently state whether prion infection has taken place. In this way we can find out more about the risks that may be associated with past treatment with blood products, which in turn will inform measures to protect public health.

The study was started by the Primary Immunodeficiency Association (PiA) and the UK Primary Immunodeficiency Network in 2006, under the leadership of Dr Matthew Helbert, Consultant Immunologist at Central Manchester University Hospitals NHS Foundation Trust. Following Dr Helbert’s retirement in 2015, management of the study transferred from Manchester to Edinburgh, initially led by Dr Anna Molesworth (PhD) for several years, and is now led by Professor Richard Knight working with the team at the National Creutzfeldt-Jakob Disease Research & Surveillance Centre (NCJDRSU) in Edinburgh.

To date, no primary immunodeficient patients have shown symptoms of prion disease, nor is there any evidence of prion infection in the tissues tested.

A recent project steering group report can also be found here.  Further information can be found at the links below.  Patients and their doctors are also welcome to talk with us about the study.  If you would like to find out more, please contact our study research nurse, Mrs Kudzai Karekwaivanane, and we will be happy to help.

Mrs Kudzai Karekwaivanane (Research Nurse)        Telephone: 0131 537 2128  / 07464 677118