Resources

UKPIN Statement on vCJD Risk in recipients of Human Immunoglobulin

8 November 2017

Antibody deficient patients should will often require endoscopic procedures such as bronchoscopy and GI endoscopy in order to examine for infection and potentially take biopsies looking for inflammatory or neoplastic disease. Variant CJD (vCJD) was first identified in the UK in 1996, and a total of 178 cases have been reported in the UK to date. Most of these cases have been linked to dietary exposure to bovine spongiform encephalopathy (BSE) in cattle. However three clinical vCJD cases and one case of asymptomatic infection are thought to have occurred as a result of blood-transfusion related person to person spread of infection. A further instance of asymptomatic vCJD infection has been identified in a recipient of UK-sourced clotting factors who had died from other causes [1]. All had received non-leucodepleted red cell transfusions or blood-products manufactured in 1999 or earlier from UK donors who were later diagnosed with clinical vCJD.

Many patients with antibody deficiency will be treated with replacement immunoglobulin. Each batch of immunoglobulin is made from the pooled plasma of not less than 1,000 donations. UK plasma has not been used to manufacture immunoglobulin since 1999, in view of the risk of vCJD transmission through blood and blood products [2].

Despite the low risk of transmission of vCJD, which has been further reduced by ceasing to use UK plasma for the manufacture of fractionated plasma products, it has remained standard practice within the UK, on initiating immunoglobulin replacement, to consent patients to the risk of known and unknown infectious agents, often specifically mentioning vCJD.

Guidance with regard to minimising transmission risk of vCJD in healthcare settings is provided by the Advisory Committee of Dangerous Pathogens (ACDP) [4]. No antibody deficient patients exposed to UK sourced immunoglobulin have either developed vCJD or been identified as having evidence of asymptomatic infection to date [1,3]. Moreover, no antibody deficient patients are considered at risk of vCJD as a result of their treatment with UK-sourced immunoglobulin. Patients who received UK-sourced immunoglobulin should not, as a consequence, be treated any differently from others who are not at risk, with regard to endoscopic or any other procedures. This is a view shared by the United Kingdom Primary Immunodeficiency Network (UKPIN) and the National CJD Research & Surveillance Unit (NCJDRSU), as well as with international experts in immunodeficiency [5].

[1] http://www.cjd.ed.ac.uk/sites/default/files/report25.pdf, accessed 31/10/17
[2] https://www.transfusionguidelines.org/document-library/documents/jpac-positionstatement-vcjd-may-2015/downloadfile/Position%20Statement%20on%20vCJD%20May%202015.pdf, accessed 31/10/17
[3] Helbert MR, Bangs C, Bishop M, Molesworth A, Ironside J. No evidence of asymptomatic variant CJD infection in immunodeficiency patients treated with UKsourced
immunoglobulin. Vox Sang. 2016 Apr;110(3):282-4
[4] https://www.gov.uk/government/publications/guidance-from-the-acdp-tse-riskmanagement-subgroup-formerly-tse-working-group, accessed 31/10/17
[5] http://www.aaaai.org/ask-the-expert/creutzfeld-jakob, accessed 31/10/17

Immunoglobulin Product Choice for Patients with Primary Immunodeficiency

UKPIN seeks to ensure the highest quality of service and support for patients with primary immunodeficiencies. In pursuit of this:

  1. Patients with primary antibody deficiency should be offered treatment with either intravenous or subcutaneous immunoglobulin.
  2. UK PIN recognises that patients with primary antibody deficiencies require life-long therapy that is acceptable and tolerable, and that the issues of immunoglobulin product choice therefore differ from other immunoglobulin uses.
  3. Patients should be given the option of undertaking treatment with immunoglobulin replacement therapy in a specialist centre, at their local hospital or at home if appropriate. This should be based on patient need and not on cost.
  4. Patients may be intolerant of a particular product. Product choice is therefore critical for the patient and their treating clinician in order to ensure appropriate and safe therapy.
  5. Patients already established on a particular product must not have their product changed for non-clinical reasons and patients being treated outside of recognised PID Centres must not have changes in product without this being discussed with the treating Immunologist. This is recognised by other international guidelines.
  6. UKPIN recognises that there may be fluctuations in the supply of particular products and endorses, as best practice, maintenance of a diversity of available immunoglobulin preparations in order to minimise disruption in the event of withdrawal or interruption of supply of a particular product.
  7. Patients with primary antibody deficiencies should have as wide a choice as possible of intra-venous and subcutaneous immunoglobulins available for long term use. The availability of a minimum of 4 different intravenous immunoglobulin and subcutaneous (preferably equivalent) products is recommended.
  8. Although UK PIN recognises the need for cost-effective drug administration, and endorses the Guidelines on Immunoglobulin use issued by the Department of Health, safety of immunoglobulin products must be of paramount importance.

Who Does What in Clinical Immunology 2014

The idea behind this project was to map out what people do to provide the infrastructure that supports our clinical specialty. We hoped that this would be a useful fact-finding exercise and that it would provide a starting point for conversations about participation and succession planning in various areas. An amazing 123 people have signed up - 52 adult consultant immunologists, 32 paediatric consultants, 32 nurses and 7 consultant clinical scientists. Thank you to everyone! 

With so many people contributing, the spreadsheet is a little unwieldy. The data is compiled firstly by professional specialty, and then by CCT date where known, and I have tried to group responsibilities together. The nurses’ data is on a separate sheet. Attributions were initially restricted it to national responsibilities, but it has grown to include network data where provided. Please accept my sincere apologies for any errors and inevitable omissions. 

There has been enthusiasm for making this information available on the web – thank you to UKPIN for ageeing to host it. The plan is to update this annually, with a census date of 1st October. Saul Faust (s.faust@soton.ac.uk) has agreed to coordinate the paediatric consultant component, and Emily Carne (Emily.Carne@wales.nhs.uk) is coordinating the nurse component. 

I hope that you find the spreadsheet useful. I think that it highlights the tremendous diversity in our specialty (as well as some of the overlap!). If you have any comments or suggestions, please don’t hesitate to contact me directly: s.e.marshall@dundee.ac.uk

Best wishes,
Sara Marshall


Networks in the UK

The table in the document below demonstrates the immunology centres in the UK and how they network with other centres in the UK. Members can upload and view documents from their and other networks in the networks forum.


An Introduction for Managers

While severe combined immunodeficiencies and related disorders have received funding through national specialist commissioning arrangements, services for other primary immunodeficiencies have developed largely due to individual effort, often expanding in a relatively unstructured and inadequately resourced way. The central message of this publication – that adults and children suffering from these disordersshould receive specialist care from health professionals appropriately trained in their management – is clearly set out and is fully endorsed by UKPIN.

This document is consistent with, broadens and explains the information provided in the National Specialised Definitions Set (Specialised Immunology, number 16).

Dr. Phil Wood
UKPIN

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