Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient.

TitlePreclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient.
Publication TypeJournal Article
Year of Publication2004
AuthorsPeden AH, Head MW, Ritchie DL, Bell JE, Ironside JW
Pagination527 - 9
Date Published2004
ISBN Number1474-547X
Other Numbers2985213r, l0s, 0053266
Keywords*Blood Transfusion/ae [Adverse Effects], *Creutzfeldt-Jakob Syndrome/tm [Transmission], *PrPSc Proteins/ge [Genetics], Codon/ge [Genetics], Creutzfeldt-Jakob Syndrome/di [Diagnosis], Creutzfeldt-Jakob Syndrome/me [Metabolism], Heterozygote, Humans, PrPSc Proteins/bl [Blood], PrPSc Proteins/ip [Isolation & Purification]

We report a case of preclinical variant Creutzfeldt-Jakob disease (vCJD) in a patient who died from a non-neurological disorder 5 years after receiving a blood transfusion from a donor who subsequently developed vCJD. Protease-resistant prion protein (PrP(res)) was detected by western blot, paraffin-embedded tissue blot, and immunohistochemistry in the spleen, but not in the brain. Immunohistochemistry for prion protein was also positive in a cervical lymph node. The patient was a heterozygote at codon 129 of PRNP, suggesting that susceptibility to vCJD infection is not confined to the methionine homozygous PRNP genotype. These findings have major implications for future estimates and surveillance of vCJD in the UK.

Notify Library Reference ID4033

Related Incidents