Variant Creutzfeldt–Jakob disease Ironside JW., Haemophilia. 2010 Jul;16 Suppl 5:175-80

REVIEW ARTICLE

Variant Creutzfeldt–Jakob disease

J. W. IRONSIDE

National Creutzfeldt–Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK Correspondence to James W. Ironside, Professor of Clinical Neuropathology and Honorary Consultant Neuropathologist, National Creutzfeldt–Jakob Disease Surveillance Unit, Division of Pathology, School of Molecular and Clinical Medicine, University of Edinburgh, Western General Hospital, Edinburgh, UK. Tel.: +44 131 537 1980; fax: +44 131 343 1404; e-mail: james.ironside@ed.ac.uk Copyright © 2010 Blackwell Publishing Ltd

KEYWORDS blood • haemophilia • plasma • prion • transfusion • variant CJD

ABSTRACT Summary.

Variant Creutzfeldt–Jakob disease (CJD) is an emerging form of human prion disease caused by oral exposure to the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, but smaller numbers of cases have been identified in 10 other countries worldwide. All confirmed cases belong to a single genetic subgroup defined by methionine homozygosity at codon 129 in the prion protein gene. Variant CJD has a widespread distribution of infectivity in the body, involving lymphoid tissues during at least the latter part of the incubation period. This is unlike other forms of human prion disease, and raised concerns that the transmissible agent might also be present in blood. To date, four probable cases of variant CJD infection have been identified following transfusion of packed red blood cells from asymptomatic donors who subsequently died from variant CJD. Recently, one case of likely transmission of variant CJD infection by UK factor VIII (FVIII) concentrates has been reported in an elderly haemophilic patient in the UK, who had been treated with FVIII produced from pooled plasma to which a donor who subsequently died from variant CJD had contributed. The recipient showed no signs or symptoms of variant CJD during life, but evidence of variant CJD infection was detected in his spleen following a postmortem examination. Continued surveillance is required to investigate the prevalence of secondary variant CJD infection in other patients with bleeding disorders who have been treated with UK-sourced pooled plasma products.

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Accepted after revision 23 April 2010

DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1365-2516.2010.02317.x About DOI

http://www3.interscience.wiley.com/journal/123553115/abstract?CRETRY=1&SRETRY=0


Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html


Sunday, August 10, 2008

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


Saturday, July 17, 2010

TSE Road map 2 A Strategy paper on TSE, a road to no where

Brussels, 16.7.2010 COM(2010)384 final COMMUNICATION FROM THE COMMISSION TO THE EUROPEAN PARLIAMENT AND THE COUNCIL

http://transmissiblespongiformencephalopathy.blogspot.com/2010/07/tse-road-map-2-strategy-paper-on-tse.html


Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/nosocomial-transmission-of-sporadic.html


Tuesday, July 13, 2010 CJD U.K. PARLIAMENT 12 July 2010 : Column 472W

The United Kingdom Parliament

http://vcjdtransfusion.blogspot.com/2010/07/cjd-uk-parliament-12-july-2010-column.html


Thursday, July 08, 2010

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html


Thursday, May 27, 2010

Guidance for Industry: Revised Preventive Measures to Reduce Possible Risk of Transmission of CJD and vCJD by blood and blood products; Availability

[Federal Register: May 27, 2010 (Volume 75, Number 102)] [Notices] [Page 29768-29769] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr27my10-66]

http://vcjdtransfusion.blogspot.com/2010/05/guidance-for-industry-revised.html


Tuesday, May 11, 2010

Current risk of iatrogenic Creutzfeld-Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

http://creutzfeldt-jakob-disease.blogspot.com/2010/05/current-risk-of-iatrogenic.html


Wednesday, March 31, 2010

Atypical BSE in Cattle / position: Post Doctoral Fellow

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html


Saturday, June 12, 2010

PUBLICATION REQUEST AND FOIA REQUEST Project Number: 3625-32000-086-05 Study of Atypical Bse

http://bse-atypical.blogspot.com/2010/06/publication-request-and-foia-request.html


Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1

http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html


NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html


Tuesday, March 16, 2010

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/03/transmissible-spongiform-encephalopathy.html


Thursday, January 28, 2010

Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States

http://creutzfeldt-jakob-disease.blogspot.com/2010/01/multiorgan-detection-and.html


Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]


http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html


http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


PLEASE SEE AND LISTEN TO VIDEO WAY BACK, toward the bottom of this url ;

Monday, July 27, 2009

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

Jeff Schwann 26 year old cjd victim USA, and please tell me why USA media did not pick this video up and run it on every evening broadcast across the USA $$$

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html


CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<





Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas.


She left 6 Kids and a Husband.

The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-

Physician Discharge Summary, Parkland Hospital, Dallas Texas

Admit Date: 12/29/2009

Discharge Date: 1/20/2010 Attending

Provider: Greenberg, Benjamin Morris;

General Neurology Team:

General Neurology Team

Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence. She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically. Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.


http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8



>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


please see full text ;


Monday, March 29, 2010


Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas


http://creutzfeldt-jakob-disease.blogspot.com/2010/03/irma-linda-andablo-cjd-victim-she-died.html


CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html



Archive Number 20100405.1091 Published Date 05-APR-2010 Subject PRO/AH/EDR> Prion disease update 1010 (04)

snip...

[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101


Sunday, July 11, 2010

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/cjd-2-cases-mclennan-county-texas.html


Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010

http://cjdtexas.blogspot.com/2010/06/usa-cases-of-dpcjd-rising-with-24-cases.html


Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html


Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html


my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd


Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html


Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/


Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html


Saturday, June 19, 2010

U.S. DENIED UPGRADED BSE STATUS FROM OIE

see full text and reasons why here ;

http://usdameatexport.blogspot.com/2010/06/us-denied-upgraded-bse-status-from-oie.html


Tuesday, August 18,

2009 BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html


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

CJD U.K. PARLIAMENT 12 July 2010 : Column 472W

The United Kingdom Parliament

12 July 2010 : Column 472W—continued

CJD Sir Paul Beresford: To ask the Secretary of State for Health how many cases of (a) variant Creutzfeldt-Jakob disease and (b) sporadic Creutzfeldt-Jakob disease were diagnosed in humans in each of the last five years; and how many such cases were as a result of blood transfusions. [7793]

Anne Milton: Data on diagnosis are not held centrally for sporadic Creutzfeldt-Jakob disease (sCJD). For variant CJD (vCJD) the number of diagnoses in each year was as follows:

2005: six cases;

2006: six cases;

2007: one case;

2008: one case; and

2009: three cases.

Of these cases, two cases diagnosed in 2006 were associated with blood transfusions in 1999 or earlier from donors who later went on to develop clinical vCJD.

Data on deaths supplied by the National CJD Surveillance Unit are shown in the following table:

Deaths from sporadic Creutzfeldt-Jakob disease (sCJD) and variant Creutzfeldt-Jakob disease (vCJD) in the UK 2005-09 Year of death sCJD sCJD-blood transfusion associated vCJD vCJD-blood transfusion associated 2005 66 0 5 0

2006 69 0 4 1

2007 63 0 4 1

2008 87 0 1 0

2009 78 0 3 0

Sir Paul Beresford: To ask the Secretary of State for Health what recent research his Department has (a) commissioned and (b) undertaken on the incidence of (i) sporadic and (ii) variant Creutzfeldt-Jakob disease and the possibility of a link in cases of individuals with different genotypes. [7794]

Anne Milton: Together with the Scottish Government, the Department funds Creutzfeldt-Jakob disease (CJD) surveillance in the United Kingdom through the National CJD Surveillance Unit (NCJDSU) based in Edinburgh. The unit was set up in 1990.

All cases of definite or probable variant Creutzfeldt-Jakob disease (vCJD), where the genotype is known, have been methionine homozygous at codon 129 of the PRNP gene. As reported in the NCJDSU's 17th annual report in 2008 the unit was referred an individual who met the clinical criteria in life for possible vCJD and who was heterozygous (methionine/valine) at codon 129 of the PRNP gene. As consent for a post-mortem 12 July 2010 : Column 476W was not given in this case, the investigations which could have led to a definite or probable diagnosis could not take place.

CJD: Screening Sir Paul Beresford: To ask the Secretary of State for Health what recent assessment his Department has made of the consequences of Amorfix's decision to suspend development of its proposed blood test for variant Creutzfeldt-Jakob disease (vCJD) after the latest versions of the test did not yield positive results; and if his Department will recognise that prion filtration is the only safe and effective method for protecting red blood cells from vCJD contamination. [7946]

Anne Milton: The Department recognises the difficulties in developing an effective blood test for the abnormal prion protein associated with variant Creutzfeldt-Jakob disease (vCJD). The Department is aware of other companies, other than Amorfix, and academic institutions developing tests and will monitor progress.

The recommendation of the Advisory Committee on the Safety of Blood, Tissue and Organs (SaBTO) on the use of prion filtration remain under consideration by the Department, the clinical safety of prion filtered blood is still being assessed, and further studies of the efficacy of prion filters are in progress.

Since the theoretical risk of vCJD transmission through blood was first identified as a possibility in 1996, a series of precautionary measures to reduce the risk of vCJD transmission through the blood supply and products made by fractionating plasma, including:

From December 1997, blood components, plasma products or tissues obtained from any individual who later develops vCJD, have been withdrawn/recalled to prevent their use;

From October 1999, white blood cells (which may carry a risk of transmitting vCJD) have been reduced in all blood used for transfusion, a process known as leucodepletion or leucoreduction;

Following the report of the first possible case of transmission of vCJD by blood transfusion in December 2003, individuals who had themselves received a transfusion of blood components since January 1980 were excluded from donating blood. This took effect from April 2004; and

In July 2004, this exclusion criterion for blood donation was extended to include two new groups; (i) previously transfused platelet donors and (ii) donors who were unsure if they had previously had a blood transfusion. This now applies to donors who have been transfused anywhere in the world.

http://www.publications.parliament.uk/pa/cm201011/cmhansrd/cm100712/text/100712w0002.htm#10071232000006



Saturday, January 20, 2007

Fourth case of transfusion-associated vCJD infection in the United Kingdom

Subject: Fourth case of transfusion-associated vCJD infection in the United Kingdom Date: January 18, 2007 at 8:32 am PST

Fourth case of transfusion-associated vCJD infection in the United Kingdom

Editorial team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office

A suspected case of variant Creutzfeldt-Jakob disease (vCJD) has recently been diagnosed in a patient in the United Kingdom (UK), who received a blood transfusion from a donor who later developed vCJD [1]. This is the fourth case of probable transfusion transmission of vCJD infection in the UK. Three of the four recipients developed symptoms of vCJD. The first symptomatic case of vCJD associated with blood transfusion was identified in December 2003. This individual developed vCJD six and a half years after transfusion of red cells donated by an individual who developed symptoms of vCJD three and a half years after donation.

A second case of vCJD 'infection' was identified a few months later in a person who had received red cells from a donor who developed symptoms of vCJD 18 months after donation. This patient (the second case) died from causes unrelated to vCJD five years after transfusion. Post-mortem investigations found abnormal prion protein in the spleen and a cervical lymph node., However, prion protein was not found in the brain, and no pathological features of vCJD were found.

A third case developed symptoms of vCJD six years after receiving a transfusion of red blood cells, and died two years and eight months later. The donor of the blood involved developed vCJD about 20 months after donating it.

These three cases have been published as case reports and in the findings of the ongoing collaborative study between the National Blood Services, the National CJD Surveillance Unit, and the Office for National Statistics. This study aims to collect evidence about transmission of CJD or vCJD via the blood supply [2,3,4,5].

The new, fourth case is in a patient who developed symptoms of vCJD eight and a half years after receiving a transfusion of red blood cells from a donor who developed vCJD about 17 months after this blood was donated [1]. The donor to this case also donated the vCJD-implicated blood transfused to the third case. As for all other reported clinical vCJD cases that have been tested for genotype, this patient is a methionine homozygote at codon 129 of the prion protein gene. The patient is currently alive.

All four cases had received transfusions of non-leucodepleted red blood cells between 1996 and 1999. Since October 1999, leucocytes have been removed from all blood used for transfusion in the UK. The effect of leucodepletion on the reduction of the risk of transmission of vCJD from an infective donation is uncertain.

This fourth case of vCJD infection associated with blood transfusion further increases the level of concern about the risk of vCJD transmission between humans by blood transfusion, although much remains unknown. This reinforces the importance of the existing precautions that have been introduced to reduce the risk of transmission of vCJD infection by blood and blood products [6]. No cases of vCJD have been associated with fractionated plasma products. The small group of living recipients of vCJD-implicated blood transfusion in the UK have been informed of their potential exposure to vCJD by blood transfusion, asked to take certain precautions to reduce the risk of onward person-to-person transmission of vCJD during health care, and offered specialist neurological evaluation and advice.

This article has been adapted from reference 1

References: Health Protection Agency. Fourth case of variant CJD associated with blood transfusion (press release). Press release, 18 January 2007.

(http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm )

Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, et al. Possible transmission of variant CJD disease by blood transfusion. Lancet 2004; 363:417-21. Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004 ; 364: 527-9. Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, et al Clinical presentation and pre-mortem diagnosis of blood transfusion-associated variant CJD. Lancet 2006;368:2061-67. Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiology review study. Vox Sang. 2006;91(3):221-230. Department of Health [London]. Further precautions to protect blood supply. Press release 2004/0104, 16 March 2004.

(http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4076608&chk=MTwE%2Bl)


http://www.eurosurveillance.org/ew/2007/070118.asp#4


HPA Press Statement

18 January 2007

4th case of variant CJD infection associated with blood transfusion

A new case of variant-Creutzfeldt-Jakob disease (vCJD ) associated with a blood transfusion has recently been diagnosed.

This latest patient has been diagnosed with vCJD about nine years after receiving a blood transfusion from a donor who later went on to develop vCJD. A transfusion from the same blood donor was also associated with one of the previously identified cases. The patient is still alive and is under specialist care.

This fourth case of vCJD infection associated with blood transfusion increases the concern about the risk of vCJD transmission between humans via blood transfusion. All four cases relate to the transfusion of blood components: no cases have been reported relating to treatment with plasma products.

The patient is one of a small number (less than 30) of living individuals who are known to have received a blood transfusion in the UK from a donor who later developed vCJD. All these individuals have previously been informed of their potential exposure to vCJD and asked to take certain precautions to reduce the chance of passing on vCJD to other people via healthcare procedures, such as surgery.

The Health Protection Agency has been in contact with doctors caring for the other patients who have been exposed to blood transfusions from donors who later developed vCJD. This is to ensure that they are informed of this new development and provide access to the latest information and specialist advice about their risk due to blood transfusion.

Professor Peter Borriello, Director of the HPA's Centre for Infections said, "This new case of vCJD infection increases our concern about the risk to the small group of people who had blood transfusions from donors who unknowingly at the time of donation must have had vCJD infection. However, this new case does not change our understanding of the risk for other people in any specific way. It does however reinforce the importance of the precautions that have already been taken to reduce the risk of transmission of vCJD infection by blood."

Dr Angela Robinson, Medical Director of NHS Blood and Transplant said, "Blood transfusions are often given to save or prolong the life of patients who are very ill and the benefit of receiving a transfusion when needed must always be balanced against any possible risk. Nonetheless, our primary concern is the safety of our patients through maintaining the quality of blood used for medical treatment. Since 1997, the NBS has introduced a range of precautionary measures against the risk of vCJD."

vCJD is a rare disease, and less than 2% of the vCJD cases reported to date in the UK have been associated with blood transfusion.

Notes to Editors:

To date, there have been 66 people identified in the UK who have received vCJD implicated blood transfusions. The transfusions received by these 66 individuals were donated by eighteen different donors who were diagnosed with vCJD after their blood donation. Of these 66 people, 40 have died of illnesses other than vCJD, including one patient who was found to have evidence of vCJD in parts of their body after their death. Including the new (4th) case, 3 of these people who have received vCJD implicated blood transfusions have developed symptoms of vCJD. There are 23 people who have received vCJD implicated blood transfusions who are alive and have not been diagnosed with vCJD.

The identification of cases of variant-CJD associated with blood transfusion has depended on the Transfusion Medicine Epidemiology Review, a collaborative study between the National Blood Services, the National CJD Surveillance Unit and the Office of National Statistics. For further information about this study see Hewitt et al Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Epidemiology Review study. Vox Sanguinis 2006 91:221-230.

'Blood Transfusion' means transfusion with labile blood components (e.g. red cells, platelets, fresh frozen plasma). This latest case (and the previous three referred to) relate to transfusion of blood components and not treatment with plasma products (i.e. products that are manufactured from plasma). To date, no case of vCJD has been associated with treatment with plasma-products (e.g. clotting factors used to treat individuals with bleeding disorders such as haemophilia).

This fourth case has been classified by the National CJD Surveillance Unit ( www.cjd.ed.ac.uk ) as a 'probable' case of vCJD. Of the 158 vCJD cases that have died (data to 5 Jan 2007), all 112 that have undergone post-mortem (46 have not) have been 'confirmed' by neuropathological examination (examination of brain tissue).

The first clinical case of vCJD associated with transfusion was identified in December 2003. A case of vCJD 'infection' associated with transfusion was identified a few months later. The patient had no symptoms but evidence of infection (abnormal prion proteins) was identified in a post mortem investigation. The individual died from causes unrelated to vCJD.

Following the first case of vCJD associated with a blood transfusion in 2003, the Department of Health asked all recipients of blood transfusions not to donate blood as a precautionary measure to protect the blood supply from vCJD.

Patients who are informed that they are considered to be 'at risk' of vCJD for public health purposes are asked to take the following precautions to reduce the chance of passing on vCJD to other people: Not to donate blood, tissues or organs and To inform their healthcare providers of their 'at-risk' status so that special procedures may be arranged for certain instruments used in their healthcare (NB. Their GPs are also asked to do this.)

A range of measures have been put in place by the Department of Health to minimise the possible risk of vCJD being passed through blood:

Since 1997 all cases of vCJD that are reported to the National CJD Surveillance Unit and diagnosed as having 'probable' vCJD, result in a searc h 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.

The likelihood of a person who may be infected with vCJD going onto develop symptoms of the disease is uncertain, and may depend on individual susceptibility. It is possible that infected individuals may never develop symptoms.

For further information contact the HPA press office on 0208 327 7098/7097/6055

Specialist care for vCJD is available from The NHS National Prion Clinic, based at The Hospital for Neurology and Neurosurgery, Queen Square, London http://www.nationalprionclinic.org/


The National CJD Surveillance Unit is based at the Western General Hospital Edinburgh: www.cjd.ed.ac.uk

For further information about vCJD go to:

http://www.hpa.org.uk/infections/topics_az/cjd/menu.htm


http://www.dh.gov.uk/PolicyAndGuidan...pics/CJD/fs/en

http://www.blood.co.uk/


http://www.cjd.ed.ac.uk


http://www.nationalprionclinic.org/


http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm


http://vcjdtransfusion.blogspot.com/2007_01_01_archive.html


http://vcjdtransfusion.blogspot.com/



Further waves of vCJD predicted

22 February 2010

Dara Gantly

dara.gantly@imt.ie Variant Creutzfeldt-Jakob Disease (vCJD) remains a 'very real and continuing threat' to public health and recent developments strongly support predictions of second and third waves of long incubation vCJD, an international expert has warned.

Dr Robert Rohwer, Associate Professor of Neurology at the University of Maryland, Baltimore, told a patient safety conference in London earlier this month (February 4) that vCJD-contaminated blood posed the greatest risk for human-to-human transmission of the disease.

"Moreover, since the transmission is between humans rather than bovine to human and the exposure is by transfusion or injection rather than orally, it is expected to be at least 1,000 times more efficient than transmissions from BSE-infected food," he stated.

Dr Rohwer stressed that this was not a theoretical risk. "To date there have been five recognised cases of transfusion transmission of vCJD and one highly probable transmission from a plasma product.

"It is not known how many donors are incubating vCJD, but accumulating evidence of long incubation times in genotypes that make up approximately two thirds of the population intimates that it may be far greater than indicated by the cases detected to date."'

The incidence of vCJD in Ireland is second to that of the UK, and ahead of France - the countries hardest hit by the disease.

In December, research by Prof John Collinge of the National Prion Clinic, published in The Lancet (Vol.374. Issue 9707, p2128), described the first patient to succumb to vCJD from a previously unaffected genetic subgroup of the prion protein. This finding strengthens an earlier prediction of second and third waves of vCJD in people who were infected over the same period as those in the first wave, but whose genetics result in a longer incubation time. Two thirds of the population is in this group, and many may be spreading the infection through blood and tissue donations.

Stressing the 'significant challenges' of detecting the prion in blood, Dr Rohwer urged implementation of proven infectivity removal technologies - such as prion filtration - to protect the blood supply.

The Department of Health in Ireland has not followed the independent expert advice of the Advisory Committee on the Safety of Blood, Tissues and Organs in the UK, which has advised the Department's counterpart in Britain to start filtering blood for children.

Chief Medical Officer Dr Tony Holohan has, however, asked the Health Information and Quality Authority (HIQA) to carry out an assessment of the new technology.

Posted in News on 22 February 2010

http://www.imt.ie/news/2010/02/further_waves_of_vcjd_predicte.html


CASE REPORT

Variant Creutzfeldt-Jakob disease in a transfusion recipient: coincidence or cause?

Gurjit Chohan, Charlotte Llewelyn, Jan Mackenzie, Simon Cousens, Angus Kennedy, Robert Will, and Patricia Hewitt From the Department of Neurology, Royal Devon and Exeter Hospital, Exeter; NHS Blood and Transplant, Cambridge; the National CJD Surveillance Unit, Western General Hospital, Edinburgh; the Infectious Disease Epidemiology Unit, London School of Hygiene and Tropical Medicine, and Chelsea and Westminster Hospital, London; and NHS Blood and Transplant, Colindale, London, UK. Correspondence to Patricia Hewitt, NHS Blood and Transplant, Colindale Avenue, Colindale, London NW9 5BG, UK; e-mail: patricia.hewitt@nhsbt.nhs.uk. The TMER study was funded by the Department of Health (Grant 007/097). This is an independent report commissioned and funded by the policy research program in the Department of Health, UK. The views expressed in the publication are those of the authors and not necessarily those of the Department of Health.

Copyright © 2010 AABB

ABSTRACT

BACKGROUND: To date there have been four instances of infection transmitted through blood transfusions derived from individuals who later developed variant Creutzfeldt-Jakob disease (vCJD). The identification of further transmission of vCJD through this route would have important implications for risk assessment and public health.

STUDY DESIGN AND METHODS: Through the UK Transfusion Medicine Epidemiology Review (TMER) the fate of blood donations from individuals who develop vCJD is traced and recipients of labile components are identified. The details of recipients are cross-checked with the register of vCJD cases held at the National CJD Surveillance Unit (NCJDSU) to identify any linkage between donors and recipients. In the reverse study, when individuals with vCJD are found to have a history of blood transfusion the donors of the transfused blood components are traced and their details cross-checked with the vCJD register to identify any missed or unrecognized linkage between donors and recipients.

CASE REPORT: A case of vCJD has been identified with a history of blood transfusion in infancy. The donors who provided the components transfused cannot be identified, but a blood donor known to have donated blood to another individual who subsequently developed vCJD could have been a donor to the index case.

RESULTS: The at-risk donor is alive 20 years after the relevant donation and continued to donate for some years, until identified as at risk, with 27 other blood components issued for use in patients, none of whom are known to have developed vCJD.

CONCLUSION: Circumstantial evidence has raised the possibility that the case in this report represents a further instance of transfusion transmission of vCJD. However, detailed investigation indicates that the pattern of events may have occurred by chance and disease in this individual may have been caused by transmission of bovine spongiform encephalopathy infection, as is the presumed cause in other primary cases of vCJD.

--------------------------------------------------------------------------------

Received for publication September 11, 2009; revision received January 7, 2010, and accepted January 7, 2010.

DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1537-2995.2010.02614.x About DOI

http://www3.interscience.wiley.com/journal/123313469/abstract?CRETRY=1&SRETRY=0



Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html


Friday, February 19, 2010

Creutzfeldt-Jakob disease (CJD) biannual update (2010/1)

http://creutzfeldt-jakob-disease.blogspot.com/2010/02/creutzfeldt-jakob-disease-cjd-biannual.html


4.49 pm Clause 2 : Blood donations

Amendment 1

Moved by Baroness Masham of Ilton

1: Clause 2, page 2, line 22, at end insert "the blood supply is made safe through the implementation of prion filtration and that"

Baroness Masham of Ilton: My Lords, the noble Lord, Lord Morris of Manchester, is president of the Haemophilia Society and I am a vice-president. We both feel that blood safety is an absolute priority, particularly for the groups of people who rely on a regular supply of clean, safe blood. I congratulate the noble Lord, Lord Morris, and the noble and learned Lord, Lord Archer of Sandwell, on their tireless efforts in championing the rights of people with haemophilia.

Amendment 1 aims to make a minor change to Part 2 of the Bill regarding the measures that need to be introduced to ensure that people with haemophilia are not given contaminated blood or blood products in the future. The amendment seeks to ensure that all diseases are covered by widening the potential range of solutions to blood diseases that can be used.

The current wording of the Bill proposes that people with haemophilia are offered a blood test for a list of conditions including hepatitis B, hepatitis C, syphilis and variant Creutzfeldt-Jakob disease-variant CJD. The challenge is that at present there is not a reliable blood test for variant CJD, unlike for other viral infections and blood-borne diseases. Detecting the infective prion that causes variant CJD is extremely difficult and as yet no one has been able to develop a test that would be reliable or effective.

However, an alternative approach to a blood test has been developed to ensure that all donated blood is free from the infective prion that causes variant CJD. This approach, prion filtration, effectively cleans the blood removing all prion whether infective or not. The P-CAPT filter has been designed to work directly with the existing technologies used by the UK National Blood Service and has been CE marked since 2006, meaning that it has passed EU-wide safety and efficacy testing, as required for it to be legally used in the UK.

In October, the Government's blood safety advisory body, SaBTO-the Advisory Committee on Safety of Blood Tissues and Organs-published advice stating that there is now sufficient evidence that the P-CAPT prion reduction filter reduces infectivity and successfully cleans blood to remove the infective prions that carry variant CJD.

The haemophilia group has had a really terrible time with HIV infection, hepatitis C and variant CJD and the risk of it. We must surely do all that we can

21 Jan 2010 : Column 1181

to protect those people. I am pleased that the noble Lord, Lord Morris of Manchester, my colleague of many years over matters relating to disability, is supporting this amendment. I wish the Bill godspeed and I beg to move.

Lord Morris of Manchester: My Lords, I am most grateful to my good friend the noble Baroness, Lady Masham of Ilton, for proposing this important amendment. As she said, we have worked in close rapport for over 40 years to enhance the status and improve the well-being of chronically ill and disabled people-she made her maiden speech on the Bill I enacted in 1970-which of course makes this an evocative moment for us both.

I diverge from her only very slightly today. She said before the debate that she was sure she was pushing an open door. In fact my door is off its hinges and I was delighted to add my name to hers as a signatory of this amendment. Thus I can be brief in my response, pointing as the noble Baroness did, to the emphasis placed in my speech on 17 March on the importance of prion filtration in removing the causative agent of variant CJD.

This debate takes place against a backcloth of human suffering on a scale that most people can barely imagine. A small and stricken community of barely 5,000 people, already disabled by a rare, lifelong blood disorder, haemophilia patients have twice been infected en masse by contaminated NHS blood and blood products. Ninety-five per cent of them were infected with hepatitis C, and one in four with HIV. Of the 1,243 haemophilia patients infected with HIV, only 361-29 per cent-are still alive. The much higher number of deaths among the hepatitis C-infected patients is still increasing.

As of now, an estimated 1,974 haemophilia patients have died from being infected in the worst ever treatment disaster in the history of the National Health Service. Should anyone dispute that assessment, they should look at the finding of distinguished statisticians that the contaminated blood disaster involved the haemophilia community in a loss of life more savage in proportion to the number of people at risk than the Black Death.

It is in that context that the sombre new threat of a third scourge facing the haemophilia community must be judged. Many hundreds of haemophilia patients have now been told by the Department of Health that they were prescribed blood from donors who subsequently died of variant CJD; indeed, a post-mortem on one such victim found variant CJD in his spleen.

The amendment addresses the new scourge and plainly warrants the support of this House.

Amendment 1 agreed.

Clause 6 : Regulations, short title, commencement and extent

Amendment 2

Moved by Lord Morris of Manchester

2: Clause 6, page 4, line 5, leave out subsection (1) and insert-

"(1) Regulations made by the Secretary of State under this Act are to be made by statutory instrument.

http://www.publications.parliament.uk/pa/ld200910/ldhansrd/text/100121-0013.htm#10012120000796


http://www.publications.parliament.uk/pa/ld200910/ldhansrd/text/100121-0013.htm#10012120000812


http://www.publications.parliament.uk/pa/ld200910/ldhansrd/text/100121-0013.htm#10012120000791



MAD COW DISEASE, ALL STRAINS

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2


Thursday, July 08, 2010

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html


Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/nosocomial-transmission-of-sporadic.html


Wednesday, June 02, 2010

CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/06/cjd-annex-h-update-after-death.html


Thursday, May 27, 2010

Guidance for Industry: Revised Preventive Measures to Reduce Possible Risk of Transmission of CJD and vCJD by blood and blood products; Availability

http://vcjdtransfusion.blogspot.com/2010/05/guidance-for-industry-revised.html


Tuesday, May 11, 2010

Current risk of iatrogenic Creutzfeld-Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

http://creutzfeldt-jakob-disease.blogspot.com/2010/05/current-risk-of-iatrogenic.html


Friday, July 17, 2009

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/revision-to-pre-surgical-assessment-of.html


Tuesday, March 16, 2010

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/03/transmissible-spongiform-encephalopathy.html


Saturday, January 16, 2010

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al Evidence For CJD/TSE Transmission Via Endoscopes


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html


Sunday, January 17, 2010

Human tissue, recovered from a donor history indicated increased risk factors for Creutzfeldt-Jacob disease Lions Eye Bank

http://creutzfeldt-jakob-disease.blogspot.com/2010/01/human-tissue-recovered-from-donor.html


Thursday, January 28, 2010

Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States

http://creutzfeldt-jakob-disease.blogspot.com/2010/01/multiorgan-detection-and.html


Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html


Tuesday, August 12, 2008

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html


Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010

http://cjdtexas.blogspot.com/2010/06/usa-cases-of-dpcjd-rising-with-24-cases.html


Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/


Wednesday, February 24, 2010

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America 14th

ICID International Scientific Exchange Brochure -

http://transmissiblespongiformencephalopathy.blogspot.com/2010/02/transmissible-spongiform-encephalopathy.html


Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html


Tuesday, August 18,

2009 BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009

http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html


TSS