Research and analysis

Creutzfeldt-Jakob disease (CJD) surveillance update 2023

Published 25 July 2024

This report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob disease (CJD). The data is correct as at 31 December 2023. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU).

Monitoring of patients ‘at increased risk’ of CJD

Individuals who have been identified as ‘at increased risk’ of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.

Public health follow-up activities include mortality monitoring and, in some cases, post mortem investigations to determine whether asymptomatic individuals have been infected with the CJD agent. A number of different organisations are involved in these activities:

• UK Health Security Agency (UKHSA)
• Public Health Scotland (PHS)
• NHS Blood and Transplant (NHSBT)
• National CJD Research and Surveillance Unit (NCJDRSU)
• National Prion Clinic (NPC)
• UK Haemophilia Centre Doctors’ Organisation (UKHCDO)

The UKHSA CJD section co-ordinates the collation of data on individuals identified as ‘at increased risk’ of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations.

The UKHSA CJD section currently holds data on the following groups of patients who have been identified as ‘at increased risk’ of CJD:

• recipients of blood components from donors who subsequently developed variant Creutzfeldt-Jakob disease (vCJD)
• blood donors to individuals who later developed vCJD
• other recipients of blood components from these blood donors
• recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
• certain surgical contacts of patients diagnosed with CJD
• highly transfused recipients
• recipients of human-derived growth hormone before 1985

Data on the following risk groups are not held by UKHSA, but are held by other organisations:

• bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
• patients who could have received a dura mater graft before August 1992 (data not currently collected)
• individuals treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
• family risk of genetic prion disease (NPC)

The data from the UKHCDO is likely to be a slight underestimate of the true number of patients with bleeding disorders who received UK-sourced clotting factors (1990 to 2001), as there was incomplete reporting of identified patients by haemophilia centres to the UKHCDO database. Notified patients are given the option of removing their details from the UKHCDO database and are then removed from the ‘at increased risk’ totals.

The data on patients who received human-derived growth hormone (previously held by the Institute of Child Health (ICH) and transferred to UKHSA at the start of 2022) is also a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.

Summary of all ‘at increased risk’ groups on which data is collected (data correct as of 31 December 2023)

Overall for all ‘at increased risk’ groups, there were 4,245 individuals notified of their increased risk status and alive as of December 31 2023; this is compared to 4,321 in 2022. There was 1 new case of iatrogenic CJD in 2023 attributable to receipt of human growth derived hormone, bringing the total number of cases to 86.

‘At increased risk’ group	Identified as ‘at increased risk’	Total notified	Total notified and alive (see note 1)	Cases	Asymptomatic infections (see note 2)
Recipients of blood from donors who later developed vCJD	67	27	11	3	1
Blood donors to individuals who later developed vCJD	112	108	94	0	0
Other recipients of blood components from these donors	34	32	11	0	0
Plasma product recipients (non-bleeding disorders) who received UK sourced plasma products 1990 to 2001	2	2	2	0	0
Certain surgical contacts of patients diagnosed with CJD	339	284	217	0	0
Highly transfused recipients	3	3	1	0	0
Recipients of human derived growth hormone (see note 3)	1,883	1,883	1,347	83	0
Total for ‘at increased risk’ groups where UKHSA holds data	2,440	2,339	1,680	86	1
Patients with bleeding disorders who received UK sourced plasma products 1980 to 2001 (see notes 3 and 4)	3,633	3,288 (see note 5)	2,565	0	1
Total for all ‘at increased risk’ groups	6,073	5,627	4,245	86	2

Note 1: throughout this report the numbers reported as “Alive” refer to those not known to have died.

Note 2: an asymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained at post mortem.

Note 3: these are minimum figures. Central reporting for bleeding disorder patients is incomplete, and a small number of patients have opted out of the central UKHCDO database. A small number of ‘at increased risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of the ‘at increased risk’ growth hormone recipients have been notified. There is no central record of who has been informed.

Note 4: including a small proportion of individuals known to have been treated with UK plasma products 1980 to 2001, and presumed to have also been treated 1990 to 2001.

Note 5: these are the minimum number of people notified based on those patients who were seen for care after the notification exercise. It is likely that many more of the ‘at increased risk’ patients received their notification letter but as they were not subsequently recorded as being seen for care this cannot be confirmed.

Current status of ‘at increased risk’ groups (data correct as of 31 December 2023)

Current status

Status	Recipients	Donors	Other recipients	Highly transfused	Plasma bleeding	Plasma non-bleeding	Surgical	Growth hormone	Total
Alive (see note 1)	11	96	11	1	2,682	2	226	1,347	4,376
Dead	54	16	23	2	951	0	113	536	1,697
Total	67	112	34	3	3,633	2	339	1,883	6,073

Note 1: throughout this report the numbers reported as “Alive” refer to those not known to have died.

Notification status

Status	Recipients	Donors	Other recipients	Highly transfused	Plasma bleeding	Plasma non-bleeding	Surgical	Growth hormone	Total
Notified	27	108	32	3	3,288	2	284	1,883	5,627
Alive and notified (see note 1)	11	94	11	1	2,565	2	214	1,347	4,245

Note 1: throughout this report the numbers reported as “Alive” refer to those not known to have died.

Genotype

Genotype	Recipients	Donors	Other recipients	Highly transfused	Plasma bleeding	Plasma non-bleeding	Surgical	Growth hormone	Total
MM	11	–	–	–	5	–	–	–	16
MV	8	–	–	–	4	–	–	–	12
VV	–	–	–	–	1	–	–	–	1
Not known	48	112	34	3	3,623	2	339	1,883	6,044

Sex

Sex	Recipients	Donors	Other recipients	Highly transfused	Plasma bleeding	Plasma non-bleeding	Surgical	Growth hormone	Total
Male	19	53	16	1	3,090	2	157	1,228	4,566
Female	20	58	18	2	543	0	174	655	1,470
Not known	28	1	0	0	0	0	8	0	37

Current age band

Age	Recipients	Donors	Other recipients	Highly transfused	Plasma bleeding	Plasma non-bleeding	Surgical	Growth hormone	Total
0 to 19	0	0	0	0	0	0	0	0	0
20 to 39	0	0	2	1	672	0	18	3	696
40 to 59	4	29	5	0	1,162	2	81	935	1,291
60 to 79	5	59	4	0	730	0	85	408	1,291
80+	2	8	0	0	118	0	42	1	171
Not known	0	0	0	0	0	0	0	0	0