Variant Creutzfeldt-Jakob disease (vCJD)
Reviewer: Huy Phu Pham, M.D. (see Reviewers page)
Revised: 1 November 2011, last major update October 2011
Copyright: (c) 2007-2011, PathologyOutlines.com, Inc.
● Prion disease results from the benign form of a prion protein changing to an insoluble protease-resistant form --> formation of plaques in brain
● Caused by prion protein that assumes an abnormal configuration, which promotes change to abnormal configuration of other proteins
Three forms of classic CJD:
● Sporadic (85%)
● Acquired (through corneal transplants, pituitary gland derived growth hormone, dura mater transplants, inadequately sterilized brain electrodes)
● vCJD is human equivalent of “mad cow” disease, due to epidemic of bovine spongiform encephalopathy, possibly due to prion in cattle entering human food supply
● In 1995, vCJD was documented in UK; transmission was through food chain
● Behavioral change, cerebellar ataxia and dementia; death in 7–38 months
● vCJD is rarely transmitted by transfusions of blood components but not plasma products
● As of 2007, in UK, 66 individuals received transfusion products from donors who later developed vCJD; 3 have developed symptoms of vCJD, one had no symptoms of vCJD but evidence of abnormal prion proteins at autopsy for unrelated death
● In US, donors who have resided in UK or Europe are deferred
● Excluding transfusion recipients from donating blood is unlikely to change epidemiology of disease (Emerg Infect Dis 2007;13:89)
● United Kingdom - confirmed (Lancet 2006;368:2061)
● Possible transmission (Lancet 2004;363:417)
● Preclinical disease in patient with unrelated death (Lancet 2004;364:527)
● Epidemiologic study (Vox Sang 2006;91:221), eMedicine, Centers for Disease Control
End of Transfusion Medicine > Tranfusion-transmitted disease > Variant Creutzfeldt-Jakob disease (vCJD)
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