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Home Topics Infectious Diseases Infections A-Z Creutzfeldt-Jakob Disease (CJD) Variant CJD and blood ›  Variant CJD and plasma products

Variant CJD and plasma products

Introduction

Asymptomatic vCJD abnormal prion protein in a haemophilia patient

Introduction

Certain plasma products, manufactured using plasma from a pool of donors, some of whom who later developed variant CJD (vCJD), may have exposed people who received them to infectivity and an increased risk of developing vCJD. The level of risk is unknown, and likely to be very low. The risk in such circumstances is in addition to a general risk for many people in the UK from past exposure to the BSE agent from eating beef and beef products.

Patients with bleeding disorders [1] who have been treated with UK sourced pooled factor concentrates or antithrombin [2] between 1990 and 2001 [3] are classified as at increased risk of vCJD for public health purposes.

In 2004 patients who had received these products were informed and asked to follow special precautions to reduce the chance of any further spread of vCJD.

Please also see Information leaflets for patients and healthcare professionals.

Asymptomatic vCJD abnormal prion protein in a haemophilia patient

A person with haemophilia, who died of other causes in 2008, was later found to have evidence of the abnormal prion protein that causes vCJD in his spleen at post mortem [4]. This is the first time that vCJD abnormal prion protein has been found in a patient with haemophilia. To date, no haemophilia or bleeding disorder patients have been diagnosed with or died from clinical vCJD.

Assuming that the abnormal prion protein detected indicated a subclinical vCJD infection, this patient had four potential routes of infection; dietary exposure to BSE; surgical procedures; transfusions with several units of red cells; and treatment with large amounts of UK sourced Factor VIII.  A risk assessment was carried out to consider the available evidence and concluded that the most likely source of this patient's infection was treatment with UK sourced clotting factors [5].

This haemophilia patient had been treated in the 1990s with over 390,000 units of UK-sourced Factor VIII.

Notes

1. Defined here as congenital and acquired haemophilia (Haemophilia A and Haemophilia B), Von Willebrand Disease, other congenital bleeding disorders and congenital antithrombin III deficiency.

2. Defined here as clotting factors and antithrombin made from pooled plasma. These include factor VIII, factor IX, factor VII, factor XI, factor XIII and prothrombin complex concentrates as well as antithrombin.

3. The start date of 1990 is the date from which it is thought that significant blood infectivity could have been present in the UK donor population from consumption of BSE contaminated beef. The end date of December 2001 is the last possible expiry date of any product manufactured by the UK fractionators that was sourced from UK donors until 1998.

4. Health Protection Report, 20 February 2009, Volume 3, No 7

5. vCJD risk assessment calculations for a patient with multiple routes of exposure. Department of Health, 9 June 2009 [external link]


Last reviewed: 31 December 2013