Hostname: page-component-cd9895bd7-jn8rn Total loading time: 0 Render date: 2024-12-30T22:06:31.088Z Has data issue: false hasContentIssue false
  • English
  • Français

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.
Get access Rights & Permissions [Opens in a new window]

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Brubaker, DB. Human posttransfusion graft-versus-host-disease. Vox Sanguinis 1983; 45: 401–420.Google ScholarPubMed
2.Anderson, KC, Weinstein, HJ. Transfusion-associated graft versus-host disease. New Eng J Med 1990; 323: 315–321.CrossRefGoogle ScholarPubMed
3.Greenbaum, BH. Transfusion-associated graft-versus-host disease: historical perspectives, incidence, and current use ofirradiated blood products. J Clin Oncol 1991; 9: 1889–1902.CrossRefGoogle ScholarPubMed
4.Hathaway, WE, Githens, JA, Blackburn, JR, Fulginiti, V, Kempe, CH. Aplastic anaemia histiocytosis and erythroderma in immunologically deficient children. New Engl J Med 1965; 273: 953–955.CrossRefGoogle ScholarPubMed
5.Parkman, R, Mosier, D, Umansky, I, Cochran, W, Carpenter, CB, Rosen, FS. Graft versus host disease after intra-uterine and exchange transfusions for hemolytic disease of the newborn. New Eng J Med 1974; 290: 359–363.CrossRefGoogle Scholar
6.Seemayer, TA, Bolande, RP. Thymic involution mimicking thymic dysplasia. A consequence of transfusion induced graft versus host disease in a premature infant. Arch Path Lab Med 1980; 104: 141–144.Google Scholar
7.Hathaway, WE, Fuginiti, VA, Pierce, CW, Githens, JH, Pearlman, DS, Muschenheim, F, Kempe, CH. Graft vs. host reaction following a single blood transfusion. J Am Med Assoc 1967; 201: 1015–1020.CrossRefGoogle ScholarPubMed
8.Park, BH, Goon, RA, Gate, J, Burke, B. Fatal graft-versus-host reaction following transfusion of allogeneic blood and plasma in infants with combined immunodeficiency. Transplant Proc 1974; 6: 385–387.Google ScholarPubMed
9.Ford, JM, Lucey, JJ, Cullen, MH, Tobias, JH, Lister, TA. Fatal graft-versus-host disease following transfusion of granulocytes from normal donors. Lancet 1976; 11: 1167.CrossRefGoogle Scholar
10.Burns, LJ, Westberg, MW, Burns, CF, Klassen, LW, Goeken, NE, Ray, TL, MacFarlane, DE. Acute graft versus host disease resulting from normal blood transfusion. Acta Haemat 1984; 71: 270–276.CrossRefGoogle Scholar
11.Sakatibara, T, Juji, T.Post-transfusion graft-versus-host disease after open heart surgery. Lancet 1986; 11: 1099. [Letter]CrossRefGoogle Scholar
12.Brouard, J, Morin, M, Borel, B, Laloum, D, Mandard, JC, Duhamel, JF, Venezia, R. Graft versus host reaction complicating DiGeorge syndrome. Arch Fr Pediatr 1985; 42: 853–855.Google Scholar
13.Wintergerst, U, Meyer, U, Remberger, K, Belohradsky, BH. Graft-versus-host reaction in an infant with DiGeorge syndrome. Monatsschr Kinderheilkd 1989; 137: 345–347.Google Scholar
14.Washington, K, Gossage, DL, Gottfried, MR. Pathology of the liver in severe combined immunodeficiency and DiGeorge syndrome. Paediatr Pathol 1993; 13: 485–504.CrossRefGoogle ScholarPubMed
15.DiGeorge, AM. Discussions on a new concept of the cellular basis of immunology. J Pediatr 1965; 67: 907. [Abstract]Google Scholar
16.Driscoll, DA, Salvin, J, Sellinger, B, Budarf, ML, McDonald-McGinn, DM, Zackai, EH, Emanuel, BS. Prevalence of 22q 11 microdeletions in DiGeorge and velocardiofacial syndromes: implications for genetic counselling and prenatal diagnosis. J Med Genet 1993; 30: 813–817.CrossRefGoogle Scholar
17.Conley, ME, Beckwith, JB, Mancer, JFK, Tenckhoff, L. The spectrum of the DiGeorge syndrome. J Pediatr 1979; 94: 883–890.CrossRefGoogle ScholarPubMed
18.Wilson, DI, Burn, J, Scambler, P, Goodship, J. DiGeorge syndrome: part of CATCH 22. J Med Genet 1993; 30: 852–856.CrossRefGoogle ScholarPubMed
19.Muller, W, Peter, HH, Wilken, M, Juppner, H, Kallfelz, HC, Krohn, HP, Miller, K, Rieger, CHL. The DiGeorge syndrome I. Clinical evaluation and course of partial and complete forms of the syndrome. Clin Immunol Immunopathol 1988; 147: 496–502.Google ScholarPubMed
20.Moerman, P, Dumoulin, M, Lauweryns, J, van der Haewaert, LG. Interrupted aortic arch syndrome. Br Heart J 1987; 58: 274–278.CrossRefGoogle Scholar
21.Hong, R. The DiGeorge anomaly. In: Rosen, FS, Seligman, (eds). Immunodefiencies. Harwood Academic Publishers, 1993, pp 167–176.Google Scholar
22.Leitman, SF, Holland, PV. Irradiation of blood products. Indications and guidelines. Transfusion 1985; 25: 293–300.CrossRefGoogle ScholarPubMed
23.Webb, DKH. Irradiation in the prevention of transfusion associated graft-versus-host disease. Arch Dis Child 1995; 73: 388–389.CrossRefGoogle ScholarPubMed
24.Dinning, G, Doughty, RW, Reid, MM, Lloyd, HL. Potassium concentrations in irradiated blood. Br Med J 1991;303: 1110.CrossRefGoogle ScholarPubMed
25.Van Mierop, LHS, Kutsche, LM. Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor. Am JCardiol 1986; 58: 133–137.CrossRefGoogle ScholarPubMed
26.Radford, DJ, Thong, YH. Facial and immunological anomalies associated with tetralogy of Fallot. Int J Cardiol 1989; 22: 229–236.CrossRefGoogle ScholarPubMed
27.Kiel, EA, Drummond, WH, Barrett, DJ. Prevalence of Tlymphocyte abnormalities in infants with congenital heart disease. Am J Dis Child 1984; 138: 143–146.Google ScholarPubMed
28.Bastian, J.Law, S, Vogler, L, Lawton, A, Herrod, H, Anderson, S, Horowitz, S, Hong, R. Prediction of persistent immunodeficiency in the DiGeorge anomaly. J Pediatr 1989; 115:391–396.CrossRefGoogle ScholarPubMed
29.Roethligsburger, FJ.Dickson, WJ. Management and the Worker. Harvard University Press, Cambridge, Massachusetts, 1939.Google Scholar
30.Silverman, FN, Kuhn, JP. Caffey's Pediatric X-ray Diagnosis: An Integrated Imaging Approach. Ninth edition. Mosby Books, St Louis, 1993, pp 651–655.Google Scholar
31.Yeager, SB, Sanders, SP. Echocardiographic identification of thymic tissue in neonates with congenital heart disease. Am Heart J 1995; 129: 837–839.CrossRefGoogle ScholarPubMed
32.Hannet, I, Erkeller-Yuksel, F, Lydyard, P, Deneys, V, DeBruyere, M. Developmental and maturational changes in blood lymphocyte subpopulations. Immunol Today 1992; 13: 215–218.CrossRefGoogle ScholarPubMed