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From: TSS ()
Subject: Re: Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report
Date: December 8, 2006 at 4:38 pm PST

In Reply to: Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report posted by TSS on December 7, 2006 at 6:58 pm:

vCJD case study highlights blood transfusion risk

Friday 8th December 2006

Scientists have confirmed that Variant Creutzfeldt-Jakob disease (vCJD) can
be

passed from person to person through blood transfusion. A case study
published

in The Lancet reports on the third person known to have contracted vCJD from

blood transfusion. The patient, who has since passed away, is the first to
have

been diagnosed whilst still alive. Two others from a group of 66 people who

received prion-infected blood from donors known to have developed vCJD, died

before their illness was confirmed.

At the age of 23, the patient was given a blood transfusion from a donor who

later developed vCJD. Seven and a half years later he was referred to the
NHS

National Prion Clinic at the National Hospital for Neurology and
Neurosurgery

where his symptoms were confirmed to be caused by vCJD. The patient opted to

join the experimental MRC treatment trial Prion-1 which began in 2004, in
which

patients are given a drug called quinacrine. Sadly, he died a year later at
the age

of 32.

Professor John Collinge of the Medical Research Council Prion Unit explains:

''That three individuals from this small group of people that we

know to have been exposed through blood transfusion have

already developed vCJD infection suggests that the infection may

be efficiently passed by this route, so the risk to remaining

individuals is likely to be substantial."

''A national tonsil tissue screening study being performed by the

Health Protection Agency may soon give estimates of the

number of people who are silently infected with prions. This

information is vital for public health planning given the relative

ease with which prions seem to be passed on by blood

transfusion."

Prion infections in humans are known to have long incubation periods, a
person

could be silently infected for more than 50 years before developing symptoms
of

the disease. During this time such a carrier of infection poses a potential
risk to

others through blood transfusion and contamination of surgical and medical

instruments.

The incubation period when prions pass from human to human is thought to be

much shorter than when they pass from one species to another. As a result,

prions that enter the body through blood transfusion as opposed to eating

infected cattle meat, like BSE prions do, probably cause vCJD to develop
more

2

quickly. When blood transfusion is the source of prions, vCJD seems, based
on

these cases, to develop in as little as 6 to 7 years.

Professor Collinge concludes:

''Analysis of samples of the patient's tonsil tissue after death,

confirmed that they were infected with prions. Examining tonsil

tissue is an established way to diagnose vCJD early if there is

reason to suspect a person has prion disease. There was

uncertainty about whether this test would also be helpful in

patients infected by blood transfusion. Study of tissue from the

patient in this case study was extremely helpful in answering this

question. In fact, it is likely that prion infection could be

diagnosed by tonsil biopsy in people who are known to be at high

risk because they have received blood from an infected donor

should they wish. Although we do not yet have an effective

treatment for any form of CJD, a reliable tonsil test could allow

people with vCJD to access experimental treatments early."

Key facts

. The prions that cause vCJD are an altered form of one of the body's

own proteins. This is why the immune system fails to recognise the

danger prions represent.

. Scientists know that a person's genetic profile plays a role in their

relative risk of developing vCJD.

MRC/42/06

Press Office

Phone: 020 7637 6011

press.office@headoffice.mrc.ac.uk

Notes to editors

1. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-

Jakob disease associated with blood transfusion: a case report is

published in The Lancet volume 368.

2. Dissociation of pathological and molecular phenotype of vCJD in

transgenic human PrP heterozygous mice was published early this year

in Proceedings of the National Academy of Sciences. The paper

describes how a person's genetic profile influences their susceptibility

to prion disease.

3. The Medical Research Council (MRC) is funded by the UK tax-payer. It

aims to improve human health. The research it supports and the

scientists it trains meet the needs of the health services, the

pharmaceutical and other health-related industries and universities.

The MRC has funded work which has led to some of the most significant

discoveries and achievements in medicine in the UK. http://www.mrc.ac.uk

4. The National Prion Clinic (NPC) is based at the National Hospital for

Neurology and Neurosurgery, Queen Square, London.The NPC provides

3

diagnosis and care for patients with, or suspected of having, any form

of human prion disease. The NPC is the national referral centre for

prion disease, and works closely with the MRC Prion Unit, based at the

Institute of Neurology, Queen Square, London. An extensive research

programme seeking to promote early diagnosis and develop treatments

for this group of diseases is now supported by the first clinical trial for

prion disease in the UK.

http://www.mrc.ac.uk/consumption/groups/public/documents/content/mrc003431.pdf

TSS






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