UKHCDO releases statement on reported cases of sporadic CJD in the UK

Written by Daniel Barnes, May 17, 2017

The UKHCDO has published this leaflet following reports in an American Journal that two female patients with inherited bleeding disorders have recently been reported to have died from sporadic CJD in 2014; one was a haemophilia carrier and the other had severe von Willebrand disease. Both had been treated with blood products in the past, but there was no link between the products these two individuals received.

Sporadic CJD is a serious progressive neurological disease that appears to occur at random when the normal prion protein in the brain undergoes a spontaneous change to the abnormal form. This results in disease with no association between the reported cases.  It mainly affects people of middle age and older.  This is a different form of CJD to vCJD which some members of our community have been informed that they have been exposed to.

The occurrence of two cases in recipients of blood products appears unusual.  However, this is the first time that sporadic CJD has been reported in patients treated with clotting factor concentrate anywhere in the world, despite surveillance systems for sCJD existing for a number of years.  At this stage, it is not known whether these two cases are a coincidence or whether the disease could be related to their treatment with clotting factor. There is active surveillance both in the UK and Internationally for both sporadic and variant CJD.

Both cases of sCJD in patients who received blood products occurred in 2014 with no other cases reported since.

You can read the publication below or download the statement here:

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