During cardiac surgery for transposition of the great arteries at age 7 wee
ks, a female infant received blood, fresh frozen plasma and platelet transf
usions. Eleven days postoperatively, she developed bloody diarrhoea, fever,
an erythematous macular rash, hepatomegaly, seizures and pancytopaenia. A
clinical diagnosis of transfusion related graft-versus-host disease (GVHD)
was supported by skin histopathology. DNA polymorphism studies confirmed th
at circulating lymphocytes in peripheral blood and infiltrating cells in th
e skin were foreign in origin and were derived from transfused blood cells.
No underlying immunodeficiency was identified. Treatment with steroids, cy
closporin and antithymocyte globulin was unsuccessful and death occurred 2
months after surgery.
The features of fever, rash, diarrhoea, liver dysfunction and pancytopaenia
which characterize GVHD may mimic drug reactions or viral infection. In ad
dition to histological features on skin biopsy, DNA polymorphism studies on
skin and blood samples provide a unique and sensitive method to confirm GV
HD. Irradiation of blood products should be considered for acutely compromi
sed infants requiring urgent cardiac surgery.