The U.S. Food and Drug Administration (FDA) has issued updated guidance to reduce the transfusion-related transmission risk of Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD). CJD is a rare but fatal disease of the central nervous system, and is transmitted by prions, misfolded proteins. The sporadic form of CJD is responsible for 85-95% of cases with about one case per million individuals worldwide. About 5-15% of CJD cases are familial or genetic, and less than 1% of cases are iatrogenic via transplantation of dura mater from donors with CJD or via contaminated preparations of cadaveric pituitary human growth hormone (hGH). Variant CJD is a distinct form of the disease, sometimes called “mad cow disease” that was linked to beef products in 1992, mainly in the U.K. Cases of vCJD peaked in 2000 at 28 cases in the U.K. and decreased following the implementation of control measures; the last two deaths due to vCJD were in the U.K. in 2013 and 2016. The FDA has determined that the risk of transfusion transmission of CJD and vCJD is very low, given no cases of transfusion transmssion CJD have been documented worldwide. Four cases of transfusion transmission of vCJD are suspected, all prior to 2004 and three from blood products that were not leukoreduced. The U.S. FDA has updated the guidance to include the removal of indefinitely deferring donors for (1) time spent in the U.K. (1980-1996) or France and Ireland (1980-2001) and (2) receiving a blood transfusion in the U.K., France, and Ireland (1980-present). In addition, the FDA recommends revising donor history questionnaires (DHQs) by removing hGH from the medicinal deferral list and removing questions about blood relatives with sporadic CJD. Individuals, however, who volunteer information about a blood relative with familial CJD or that they have taken hGH should be permanently deferred. The FDA has also issued two draft guidances that are now open for comment regarding (1) donor eligibility requirements for blood pressure and pulse and (2) compliance policy for donation suitability, donor eligibility, and source plasma quarantine hold requirements.
References:
- FDA Regulatory Information. Recommendations to reduce the possible risk of transmission of Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease by blood and blood components. Guidance for Industry, updated May 2022.
- FDA Regulatory Information. Blood pressure and pulse donor eligibility requirements—compliance policy. Draft Guidance for Industry, May 2022.
- FDA Regulatory Information. Compliance policy regarding blood and blood component donation suitability, donor eligibility, and source plasma quarantine hold requirements. Draft Guidance for Industry, May 2022.
