Implementation of BSH 2020 irradiation guidelines for children with DiGeorge syndrome

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.

To understand UK use of irradiated components for paediatric CHD surgery and barriers to BSH guideline implementation.

An electronic survey distributed to the 11 UK paediatric cardiothoracic surgical centres (January-March 2024 inclusive).

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.

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.