----- Original Message -----
From: "Terry S. Singeltary Sr." <[email protected]>
To: <[email protected]>
Sent: Thursday, December 07, 2006 9:03 PM
Subject: Clinical presentation and pre-mortem diagnosis of variant
Creutzfeldt-Jakob disease associated with blood transfusion: a case report
##################### Bovine Spongiform Encephalopathy
#####################
The Lancet 2006; 368:2061-2067
DOI:10.1016/S0140-6736(06)69835-8
Articles
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob
disease associated with blood transfusion: a case report
Stephen J Wroe FRCP a b, Suvankar Pal MRCP a b, Durrenajaf Siddique MRCP a
b, Harpreet Hyare FRCR a b, Rebecca Macfarlane MRCS a b, Susan Joiner MSc b,
Jacqueline M Linehan BSc b, Sebastian Brandner MRCPath b, Jonathan DF
Wadsworth PhD b, Patricia Hewitt FRCPath c and Prof John Collinge FRS a b
Summary
Background
Concerns have been raised that variant Creutzfeldt-Jakob disease (vCJD)
might be transmissible by blood transfusion. Two cases of prion infection in
a group of known recipients of transfusion from donors who subsequently
developed vCJD were identified post-mortem and reported in 2004. Another
patient from this at-risk group developed neurological signs and was
referred to the National Prion Clinic.
Methods
The patient was admitted for investigation and details of blood transfusion
history were obtained from the National Blood Service and Health Protection
Agency; after diagnosis of vCJD, the patient was enrolled into the MRC
PRION-1 trial. When the patient died, brain and tonsil tissue were obtained
at autopsy and assessed for the presence of disease-related PrP by
immunoblotting and immunohistochemistry.
Findings
A clinical diagnosis of probable vCJD was made; tonsil biopsy was not done.
The patient received experimental therapy with quinacrine, but deteriorated
and died after a clinical course typical of vCJD. Autopsy confirmed the
diagnosis and showed prion infection of the tonsils.
Interpretation
This case of transfusion-associated vCJD infection, identified ante-mortem,
is the third instance from a group of 23 known recipients who survived at
least 5 years after receiving a transfusion from donors who subsequently
developed vCJD. The risk to the remaining recipients of such tranfusions is
probably high, and these patients should be offered specialist follow-up and
investigation. Tonsil biopsy will allow early and pre-symptomatic diagnosis
in other iatrogenically exposed individuals at high risk, as in those with
primary infection with bovine spongiform encephalopathy prions.
Affiliations
a. National Prion Clinic, National Hospital for Neurology and Neurosurgery,
Queen Square, London WC1N 3BG, UK
b. MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College London, London, UK
c. National Blood Service, London, UK
Correspondence to: Prof John Collinge
http://www.thelancet.com/journals/lancet/article/PIIS0140673606698358/abstract
TSS
#################### https://lists.aegee.org/bse-l.html
####################
From: "Terry S. Singeltary Sr." <[email protected]>
To: <[email protected]>
Sent: Thursday, December 07, 2006 9:03 PM
Subject: Clinical presentation and pre-mortem diagnosis of variant
Creutzfeldt-Jakob disease associated with blood transfusion: a case report
##################### Bovine Spongiform Encephalopathy
#####################
The Lancet 2006; 368:2061-2067
DOI:10.1016/S0140-6736(06)69835-8
Articles
Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob
disease associated with blood transfusion: a case report
Stephen J Wroe FRCP a b, Suvankar Pal MRCP a b, Durrenajaf Siddique MRCP a
b, Harpreet Hyare FRCR a b, Rebecca Macfarlane MRCS a b, Susan Joiner MSc b,
Jacqueline M Linehan BSc b, Sebastian Brandner MRCPath b, Jonathan DF
Wadsworth PhD b, Patricia Hewitt FRCPath c and Prof John Collinge FRS a b
Summary
Background
Concerns have been raised that variant Creutzfeldt-Jakob disease (vCJD)
might be transmissible by blood transfusion. Two cases of prion infection in
a group of known recipients of transfusion from donors who subsequently
developed vCJD were identified post-mortem and reported in 2004. Another
patient from this at-risk group developed neurological signs and was
referred to the National Prion Clinic.
Methods
The patient was admitted for investigation and details of blood transfusion
history were obtained from the National Blood Service and Health Protection
Agency; after diagnosis of vCJD, the patient was enrolled into the MRC
PRION-1 trial. When the patient died, brain and tonsil tissue were obtained
at autopsy and assessed for the presence of disease-related PrP by
immunoblotting and immunohistochemistry.
Findings
A clinical diagnosis of probable vCJD was made; tonsil biopsy was not done.
The patient received experimental therapy with quinacrine, but deteriorated
and died after a clinical course typical of vCJD. Autopsy confirmed the
diagnosis and showed prion infection of the tonsils.
Interpretation
This case of transfusion-associated vCJD infection, identified ante-mortem,
is the third instance from a group of 23 known recipients who survived at
least 5 years after receiving a transfusion from donors who subsequently
developed vCJD. The risk to the remaining recipients of such tranfusions is
probably high, and these patients should be offered specialist follow-up and
investigation. Tonsil biopsy will allow early and pre-symptomatic diagnosis
in other iatrogenically exposed individuals at high risk, as in those with
primary infection with bovine spongiform encephalopathy prions.
Affiliations
a. National Prion Clinic, National Hospital for Neurology and Neurosurgery,
Queen Square, London WC1N 3BG, UK
b. MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College London, London, UK
c. National Blood Service, London, UK
Correspondence to: Prof John Collinge
http://www.thelancet.com/journals/lancet/article/PIIS0140673606698358/abstract
TSS
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