Variant Creutzfeldt-Jakob disease and its transmission by blood

James Ironside, Mark Head

Research output: Contribution to journalArticlepeer-review

Abstract

Variant Creutzfeldt-Jakob disease (vCJD) is a novel acquired human prion disease resulting from human exposure to the agent causing bovine spongiform encephalopathy (BSE). vCJD differs from all other human prion diseases in that the disease-associated form of the prion protein and infectivity are present in lymphoid tissues throughout the body. Lymphoid tissues and lymphocytes are implicated in the peripheral pathogenesis of prion diseases (where infectivity may be detected during the preclinical phase of the illness), giving rise to concerns that blood and blood products may also contain infectivity, thus representing a possible source of iatrogenic spread of vCJD. These concerns have been reinforced by the recent transmission of BSE in an experimental sheep model by blood transfusion from an infected animal in the preclinical phase of the illness. Studies in other animal models suggest that most infectivity in blood may be cell-associated, with lower levels in the plasma, and there is evidence to indicate that any infectivity present may be reduced during the process of plasma fractionation. At present, the attempts to detect disease-associated prion protein and infectivity in buffy coat from vCJD patients have been negative, but these studies have been limited in size and in the sensitivity of the detection systems employed. Further studies are required to develop more sensitive means of detection of disease-associated prion protein in blood; such techniques could also be employed for screening purposes, both individually and to help ascertain more precisely the likely numbers of future cases of vCJD.

Original languageEnglish
Pages (from-to)1479-86
Number of pages8
JournalJournal of Thrombosis and Haemostasis
Volume1
Issue number7
Publication statusPublished - Jul 2003

Keywords

  • Adult
  • Aged
  • Animals
  • Blotting, Western
  • Creutzfeldt-Jakob Syndrome
  • Disease Models, Animal
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prion Diseases
  • Sheep

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