Transfusion medicine
Transfusion transmitted disease
Variant Creutzfeldt-Jakob disease (vCJD)

Author: Huy Phu Pham, M.D. (see Authors page)

Revised: 30 October 2017, last major update October 2011

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PubMed Search: Variant Creutzfeldt-Jakob disease [title] "loattrfree full text"[sb] symptoms

Cite this page: Pham, H.P. Variant Creutzfeldt-Jakob disease (vCJD) . PathologyOutlines.com website. http://pathologyoutlines.com/topic/transfusionmedcreutzfeldtjakob.html. Accessed November 23rd, 2017.
Clinical features
  • 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%)
  • Hereditary
  • 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 U.K.; transmission was through food chain

vCJD symptoms:
  • 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 U.K., 66 individuals received transfusion products from donors who later developed vCJD; 3 have developed symptoms of vCJD, 1 had no symptoms of vCJD but evidence of abnormal prion proteins at autopsy for unrelated death
  • In U.S., donors who have resided in U.K. or Europe are deferred
  • Excluding transfusion recipients from donating blood is unlikely to change epidemiology of disease (Emerg Infect Dis 2007;13:89)
Case reports