Scientists warn of first ever case of human mad cow disease

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Feb 27, 2006
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Scientists warn of first ever case of human mad cow disease from blood plasma

The first case of a person being infected with the human form of mad cow disease after receiving contaminated blood plasma has been identified by scientists.

By Patrick Hennessy and Laura Donnelly Last Updated: 11:10AM GMT 15 Feb 2009

Scientists fear there could be a second wave of the human variant of mad cow disease, which was caused by cattle being fed the remains of other cattle in the 1980s Photo: EPA The man was one of thousands of haemophiliacs who received blood plasma transfusions in the years before strict controls were brought in to eliminate the spread of variant Creutzfeldt-Jakob disease (vCJD).

Until now, scientists had maintained that the 4,000 people who may have received plasma from infected donors were at very low risk of developing the fatal brain disease. Warnings were issued to them as a "highly precautionary measure".

But the Health Protection Agency is expected to announce on Tuesday that an elderly man, who died from other causes, contracted vCJD from plasma.

Although vCJD has been transmitted by blood donations in the past, leading to three deaths, no cases of infection had ever been linked to plasma, which is used to clot blood. Scientists had believed the processing and dilution of the product before it is injected into patients significantly reduced the risks.

BSE expert Professor Hugh Pennington, Emeritus Professor of Bacteriology at Aberdeen University said the findings would have "significant implications" for thousands of people who had been given plasma before the dangers were suspected.

"This looks like pretty grim news for a group of people who have been through fire and water for so long; they have already had increased exposure to hepatitis B and HIV," he said.

Warnings were sent to 4,000 haemophiliacs, and patients suffering from other rare blood conditions in 2004 to warn them that they had had received transfusions from 200 batches of blood products at risk of contamination with vCJD. The plasma was collected from nine people who went on to develop the brain-wasting disease.

All 4,000 were advised not to give blood or donate organs and to warn doctors and dentists that they had been put at risk by the use of plasma.

To date, 164 people have died from vCJD in Britain, with most cases linked to eating meat infected with bovine spongiform encephalopathy.

Prof Pennington said details of the way the new link had been detected would be crucial in determining further investigations.

"There is a lot more we still need to know. The fact that this person is elderly, when most of the deaths from vCJD have been young people, and that they died from another cause, is another area for research," he said, suggesting that it might mean that the disease progressed more slowly in some people.

He said restrictions over blood donation, which mean anyone who has had a transfusion cannot donate, and that all plasma is now taken from stocks in the United States, meant the risks to those receiving blood or plasma now were "vanishingly low".

The brain-wasting disease vCJD was first detected in the mid 1990s. Most vCJD patients have been infected after eating BSE contaminated meat. The number of deaths peaked in 2000, when there were 28 deaths. That number has dropped to about five cases a year since 2005.

The epidemic of BSE in the 1980s and 1990s was caused by cattle being fed the remains of other cattle in the form of meat and bone meal, causing an infectious agent to spread.

More than 4 million cattle were slaughtered after almost 200,000 were infected with the fatal neurodegenerative disease.

Scientists recently warned that Britain could see a second wave of the vCJD, affecting as many as 300 people, after discovering that genetic differences can affect how long it takes a person to incubate the disease. ... lasma.html

Title a bit misleading, should have probably read something like ;

>>>Scientists warn of first ever DOCUMENTED case of human mad cow disease from blood plasma<<<

WITH the many strains or phenotypes of the sporadic CJD's, plus, the NEW TSE announced last year by Gambetti et al here in the USA, it would seem prudent to a look back at all TSE suspect donors, considering the terribly poor BSE surveillance system in the USA, and especially since all strains of BSE have been documented in North America i.e. the typical UK c-BSE strain, the H and L atypical BSE strain in Canada, and the TWO cases of H type cases in Texas and Alabama, plus the one typical c-BSE imported case in Washington. anyone that does not think the L-BSE strain is in not in the USA, considering for one thing, the infamous 'enhanced june 2004 bse surveillance' was flawed from the beginning, before it was shut down just about completely, well they would only be dreaming in my opinion. considering all these factors, there is a high likelihood, also in my opinion, that the USA blood supply has been tainted with TSE for some time.

let's take another _look back_ at the nvCJD ONLY blood recalls in the USA ;


Due to the recent discovery of two cases of vCJD blood transfusion transmission in the U.K. the American Red Cross, at the October 14 FDA TSEAC meeting, announced an acceleration of their "Look Back" study implemented in 1995. In this study all blood and blood products donated to the American Red Cross by a person who subsequently died of CJD will be traced.

This "Look Back" study is a very important one for all of our families of CJD patients, past and present. If your loved one was a blood donor or a blood recipient and you wish to participate please contact one of the names listed below.

As the wife of a regular blood donor who died of CJD in 2000 I am relieved to know that this study is being given high priority by the ARC and the CDC. I know we all certainly hope that sCJD is not a vector, however, we won't know unless this research is conducted.

The recipients of any whole blood or blood product donated by a person who subsequently died of CJD will NOT be notified unless medically appropriate notification and counseling is deemed necessary by the health care provider.

Contact Information:

Kerri Dorsey, MPH 301-738-0592 E-mail: [email protected]

Shimian Zou, PhD 301-738-0644 E-mail: [email protected]



Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

see full text ; ... pdate.html

Quick Summary for the Transmissible Spongiform Encephalopathies Advisory Committee (TSEAC) Oct 14, 2004

In the afternoon open public hearing Peter L. Page, MD, from the American Red Cross presented their "CJD Lookback Study"; Michael Fitzpatrick, PhD, of America's Blood Centers requested FDA consider an "exit strategy" for CJD deferrals. Other presentations and comments were made by: Robert Rohwer, PhD, VA Hospital Baltimore; Merlyn Sayers, MD, PhD, Carter Blood Care; Jonathan Goldsmith, MD, Immune Deficiency Foundation and David Cavenaugh, Committee of Ten Thousand. Three written submissions to the meeting record were received including a copy of a letter from a woman in the UK to her husband's consultant regarding vCJD and questions from her for this meeting, an e-mail from Terry Singletary and an e-mail from Barbara Sachau. The major topic of the meeting was a discussion entitled, "Consideration of Current FDA-Recommended Safeguards to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease (CJD) and Variant Creutzfeldt-Jakob Disease (vCJD) by Blood and Blood Products". In preparation for discussion of this topic the Committee listened to the following presentations:

snip... ... inutes.pdf

From: Terry S. Singeltary Sr.

To: [email protected]

Cc: [email protected] ; [email protected]

Sent: Friday, December 01, 2006 2:59 PM

Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION

----- Original Message -----

From: Terry S. Singeltary Sr.

To: [email protected]

Cc: [email protected] ; [email protected]

Sent: Wednesday, November 29, 2006 1:24 PM Subject: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION]

November 29, 2006

Greetings FDA, DHH, Dr. Freas, and Dr. Harvey et al,

snip... see full text ;

Sunday, February 15, 2009

Scientists warn of first ever case of human mad cow disease from blood plasma ... se-of.html

vCJD abnormal prion protein found in a patient with haemophilia at post mortem

17 February 2009

Evidence of infection with the agent (abnormal prion protein) that causes variant Creutzfeldt-Jakob Disease (vCJD) has been found at post mortem in the spleen of a person with haemophilia.

The patient, who was over 70 years old, died of a condition unrelated to vCJD and had shown no symptoms of vCJD or any other neurological condition prior to his death. The vCJD abnormal prion protein was only identified during post mortem research tests.

The Health Protection Agency is working with the UK Haemophilia Centre Doctors Organisation to ensure all patients with bleeding disorders are made aware of this preliminary information which is being further investigated. This new finding will not change the way patients with haemophilia are cared for or treated.

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. It is known that the patient had been treated with several batches of UK sourced clotting factors before 1999, which is when measures to improve the safety of blood in relation to vCJD were introduced. The patient's treatment had included one batch of Factor VIII that was manufactured using plasma from a donor who went on to develop symptoms of vCJD six months after donating the plasma in 1996.

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. This new finding, however, does not change the public health vCJD 'at risk' status of patients with bleeding disorders.

Haemophilia patients have previously been informed by their doctors of their possible increased risk of exposure to vCJD via clotting factors. In 2004 all patients with bleeding disorders who had been treated with UK-sourced pooled plasma products between 1980 and 2001 were told that, owing to potential vCJD infectivity from these products they were to be classified as at-risk of vCJD for public health purposes.

Professor Mike Catchpole, Director of the Health Protection Agency's Centre for Infections, said:

"This new finding may indicate that what was until now a theoretical risk may be an actual risk to certain individuals who have received blood plasma products, although the risk could still be quite low. We recognise that this finding will be of concern for persons with haemophilia who will be awaiting the completion of the ongoing investigations and their interpretation.

The priority is to ensure that patients are informed of this development and have access to the latest information and specialist advice from their own haemophilia centre doctor as soon as possible.

"This finding does not change our understanding of the risk from vCJD for other people in any specific way. But it does reinforce the importance of the precautionary measures that have been taken over the years.

"Since the risk of vCJD transmission through blood was first considered, a number of precautionary measures have been introduced to minimise the risk from the UK blood supply. UK plasma has not been used for the manufacture of clotting factors since 1999 and synthetic clotting factors are provided for all patients for whom they are suitable."


Notes for editors 1) The post-mortem tests were carried out as part of a research study jointly coordinated by the UK Haemophilia Centre Doctors Organisation and the National CJD Surveillance Unit. The study was commissioned in 2001 and is ongoing.

2) The likelihood of a person who is infected with the vCJD abnormal prion protein going on to develop symptoms of the disease is uncertain and may depend on individual susceptibility. It is possible that infected individuals may never develop symptoms.

3) Haemophilia is a genetic blood condition in which an essential clotting factor is either partly or completely missing. This causes a person with haemophilia to bleed for longer than normal. Treatment for haemophilia is usually by replacing the missing clotting factor (factor VIII) through regular injections which helps the blood to clot and minimises the likelihood of long term joint damage.

4) In 2004 all patients with bleeding disorders who had been treated with UK-sourced pooled plasma products (e.g. clotting factors for individuals with haemophilia) between 1980 and 2001 were told that, owing to potential vCJD infectivity from these products, they would be classified as at-risk of vCJD for public health purposes.

The start date of 1980 is thought to be the earliest date the agent (abnormal prion protein), that causes BSE in cattle and vCJD in humans, could have entered the food chain. The end date of 2001 is the last possible expiry date of any product manufactured by UK fractionators that had been sourced from UK donors up until 1998.

5) The government introduced a number of measures from 1997 onwards to safeguard blood and plasma supplies.

Since 1997 all cases of vCJD that are reported to the National CJD Surveillance Unit and diagnosed as having 'probable' vCJD, result in a search of the UK Blood Services blood donor records. If the patient has donated blood, any unused parts of that blood are immediately removed from stock. The fate of all used components of blood from the donor is traced, and surviving recipients informed of their risk. In July 1998, the Department of Health announced that plasma for the manufacture of blood products, such as clotting factors, would be obtained from non-UK sources. Since October 1999, white blood cells (which may carry the greatest risk of transmitting vCJD) have been removed from all blood used for transfusion. In August 2002 the Department of Health announced that fresh frozen plasma for treating babies and young children born after 1 January 1996 would be obtained from the USA, extended to all children under 16 years of age (Summer 2005). In December 2002, the Department of Health completed its purchase of the largest remaining independent US plasma collector, Life Resources Incorporated. This secures long-term supplies of non-UK blood plasma for the benefit of NHS patients. Since April 2004, blood donations have not been accepted from people who have themselves received a blood transfusion in the UK since 1980. This has been extended to include apheresis donors and donors who are unsure if they had previously had a blood transfusion (August 2004). Since late 2005, blood donations have not been accepted from donors whose blood was transfused to patients who later developed vCJD. The UK Blood Services continue to promote the appropriate use of blood and tissues and alternatives throughout the NHS.

6) Specialist advice and care concerning vCJD is available from:

The National CJD Surveillance Unit, based at the Western General Hospital Edinburgh: The NHS National Prion Clinic, based at The Hospital for Neurology and Neurosurgery, Queen Square, London

7) For further information about vCJD go to: ... /CJD/fs/en

8) For Health Protection Agency media enquiries please contact the Agency's Centre for Infections Press Office on:

Kate Swan 020 8327 7097

Alexandra Baker 0208 327 7098

Louise Brown 020 8327 7080

George Fletcher 020 8327 6690 ... 1252394302 ... nd-in.html

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

Sunday, February 15, 2009
Scientists warn of first ever case of human mad cow disease from blood plasma ... se-of.html

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