Implementation of
BSH
2020 irradiation guidelines for children with
DiGeorge
syndrome
Oliver Firth, Philip Arnold, Andrew R. Gennery, Robert Johnson, Sara Mele, Tracey Shackleton, John Grant‐Casey, Theodora Foukaneli, Helen V. New Abstract
Background
Transfusion‐associated graft‐versus‐host disease (Ta‐GvHD) is a rare, usually fatal complication of transfusing cellular components, prevented by irradiation. Congenital heart disease (CHD), particularly DiGeorge syndrome (del22q), may be associated with severe T‐cell immunodeficiency and Ta‐GvHD risk. British Society for Haematology guidelines (BSH, 2020) recommended targeted use of irradiated components for CHD based on immunological assessment; guideline adherence was unknown.
Objectives
To understand UK use of irradiated components for paediatric CHD surgery and barriers to BSH guideline implementation.
Methods
An electronic survey distributed to the 11
Results
Of the 11 centres, 9 (82%) responded. Naïve T‐cell enumeration, the BSH recommended test, was available to 8/9 (89%), but used in 4/9 (44%). 4/9 (44%) and 5/9 (56%) centres were willing to provide non‐irradiated components to neonates and children >28 days of age. Of these, 1/4 (25%), 1/5 (20%), and 4/5 (80%) (for neonates, young children, and children from 2 years) required criteria additional to BSH guidelines. 8/9 (89%) centres reported ongoing barriers to guideline implementation, particularly challenges around pathway development and timely naïve T‐cell enumeration.
Conclusion
Overuse of irradiated components in surgery for patients with congenital heart disease is high: only 1/3 of paediatric cardiac centres adhered to BSH guidance. Reasons included difficulties in immunological diagnostic pathway development. Collaboration between paediatric cardiothoracic, transfusion and immunology teams is required to address this.