Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia

ORIGINAL ARTICLE

Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia

A. PEDEN*, L. McCARDLE*, M. W. HEAD*, S. LOVE†, H. J. T. WARD*, S. N. COUSENS‡, D. M. KEELING§, C. M. MILLAR¶, F. G. H. HILL** and J. W. IRONSIDE* *National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Edinburgh ; †Department of Neuropathology, Frenchay Hospital, Bristol ; ‡London School of Hygiene and Tropical Medicine, London ; §Oxford Haemophilia and Thrombosis Centre, Churchill Hospital, Oxford ; ¶Department of Haematology, Imperial College London, London ; and **Department of Haematology, Birmingham Children's Hospital, Birmingham, UK Correspondence to Prof. James W. Ironside, National Creutzfeldt–Jakob Disease Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK. Tel.: +44 131 537 3109; fax: +44 131 343 1404; e-mail: james.ironside@ed.ac.uk Copyright © 2010 Blackwell Publishing Ltd

KEYWORDS haemophilia • plasma • prion protein • spleen • vCJD

ABSTRACT

Summary.

All UK patients with bleeding disorders treated with any UK-sourced pooled factor concentrates between 1980 and 2001 have been informed that they may be at an increased risk of infection with variant Creutzfeldt–Jakob disease (vCJD). We describe a study to detect disease-associated, protease-resistant prion protein (PrPres) in 17 neurologically aysmptomatic patients with haemophilia considered to be at increased risk of vCJD. Materials from 11 autopsy and seven biopsy cases were analysed for PrPres. The tissues available from each case were variable, ranging from a single biopsy sample to a wide range of autopsy tissues. A single specimen from the spleen of one autopsy case gave a strong positive result on repeated testing for PrPres by Western blot analysis. This tissue came from a 73-year-old male patient with no history of neurological disease, who was heterozygous (methionine/valine) at codon 129 in the prion protein gene. He had received over 9000 units of factor VIII concentrate prepared from plasma pools known to include donations from a vCJD-infected donor, and some 400 000 units not known to include donations from vCJD-infected donors. He had also received 14 units of red blood cells and had undergone several surgical and invasive endoscopic procedures. Estimates of the relative risks of exposure through diet, surgery, endoscopy, blood transfusion and receipt of UK-sourced plasma products suggest that by far the most likely route of infection in this patient was receipt of UK plasma products.

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Accepted after revision 29 November 2009


DIGITAL OBJECT IDENTIFIER (DOI) 10.1111/j.1365-2516.2009.02181.x About DOI



http://www3.interscience.wiley.com/journal/123238033/abstract




Friday, November 20, 2009


SaBTO Advisory Committee on the Safety of Blood, Tissues and Organs Summary of the Eighth Meeting, 27 October 2009


http://vcjdtransfusion.blogspot.com/2009/11/sabto-advisory-committee-on-safety-of.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



Monday, August 17, 2009

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.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, 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




Saturday, January 16, 2010


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





R.I.P. MOM hvCJD confirmed DECEMBER 14, 1997

Monday, December 14, 2009

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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




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