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Transfusion associated graft-versus-host disease in DiGeorge syndrome—index case report with survey of screening procedures and use of irradiated blood components

Published online by Cambridge University Press:  19 August 2008

Rodney C. G. Franklin ,
Obed Onuzo ,
Paul A. Miller ,
David Goldblatt  and
David Cummins
Rodney C. G. Franklin*
Affiliation:
Institute of Child Health and the Great Ormond Street Hospital for ChildrenLondon
Obed Onuzo
Affiliation:
Institute of Child Health and the Great Ormond Street Hospital for ChildrenLondon
Paul A. Miller
Affiliation:
Institute of Child Health and the Great Ormond Street Hospital for ChildrenLondon
David Goldblatt
Affiliation:
Middlesex and Immunobiology Unit, Institute of Child Health and the Great Ormond Street Hospital for Children, London
David Cummins
Affiliation:
Departments of Paediatric Cardiology and Haematology, Harefield Hospital, Harefield, Institute of Child Health and the Great Ormond Street Hospital for Children, London
*
Dr. Rodney Franklin, Consultant Paediatric Cardiologist, Harefield Hospital, Middlesex UB9 6JH, United Kingdom. Tel. 01895 828554; Fax. 01895 824983.
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Summary

Most patients with DiGeorge syndrome require surgery to their heart defect during infancy and are at risk of developing transfusion-associated graft-versus-host disease if non-irradiated blood is used perioperatively. Because of its wide clinical variability, DiGeorge syndrome may not be recognized before surgery and thus a high level of vigilance is required. An infant is described who developed fatal graft-versus-host disease after receiving non-irradiated blood components during surgery for classical tetralogy of Fallot; the postoperative clinical course and autopsy findings were consistent with underlying DiGeorge syndrome. An informal survey of the 18 pediatric cardiology units in the United Kingdom and the Republic of Ireland showed no consensus as to which patients should be screened for DiGeorge syndrome, which screening test to use and to whom irradiated blood components should or should not be given to prevent transfusion associated graft-versus-host disease. Assessment of T-cell numbers appears to be the preferred screening method to determine those who might benefit from receiving such therapy.

Keywords

Graft-versus-host diseaseblood transfusionDiGeorge syndrome
Type
Original Manuscripts
Information
Cardiology in the Young , Volume 6 , Issue 3 , July 1996 , pp. 222 - 227
Copyright
Copyright © Cambridge University Press 1996

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