Transfusion-Associated Graft-Versus-Host Disease Confirmed by Human Leukocyte Antigen Typing in a Patient with Severe Combined Immunodeficiency and Review of the Literature

Background: Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare, but often lethal complication of cellular blood componenttransfusion that produces a graft-versus-host clinical manifestation in immunodeficient patients. We report a patient who developed TA-GVHDand provide a review of the literature.Method: We report an infant with severe combined immunodeficiency (SCID) who developed TA-GVHD. The patient received a nonirradiated,packed erythrocyte cell suspension and platelet transfusions from unrelated donors, before the diagnosis of SCID. The patient manifested symptomsand signs of TA-GVHD (fever, skin rash, diarrhea, icterus, eosinophilia and bone marrow failure) 3-weeks after blood product transfusions.Result: Immunology investigation was consistent with T– B– NK+ SCID. The recto-sigmoid biopsies confirmed the gold standard features of grade-IIacute GVHD. HLA typing of the patient and his parents showed that the patient has an extra-parental-allele of major histocompatibility complex(MHC) class I B*53. He received high doses of methylprednisolone, IVIG and ursodeoxycholic acid, but he had progressive hyperbilirubinemiaand bone marrow failure, then he developed candidemia and pseudomonas aeruginosa sepsis and multiorgan failure then he died.Discussion / Conclusion: SCID is one of several risks for TA-GVHD. TA-GVHD develops when transfused blood-derived immunocompetent,alloreactive T lymphocytes able to engraft in the recipient’s lymphoid tissues that fail to reject them. Those lymphocytes mediate immuneresponse causing damage and dysfunction of the skin, gastrointestinal tract, liver and bone marrow failure. Our patient showed all featuresof TA-GVHD that was complicated by fulminant sepsis and multiorgan failure despite aggressive management. The diagnosis of this lethalcondition needs high index of suspicion and the transfusion history must be questioned in all immunodeficiency patients. The disease is fulminateand rapidly fatal in majority of patients even with aggressive treatment, while irradiation of blood products that to be given to recipients atrisk is the preventive method of choice.