title: Transfusion-associated graft-versus-host disease in severe combined immunodeficiency.
abstract: Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of cellular blood component transfusion that produces a graft-versus-host clinical picture with concomitant bone marrow aplasia. We report the case of 2 patients with severe combined immunodeficiency (SCID) who developed TA-GvHD. Both patients had been given nonirradiated erythrocyte suspension before the diagnosis of SCID. Although one of them was aged 12 months, he had still not been diagnosed as having severe T-cell deficiency at the time of transfusion. Both patients presented similar signs and symptoms (fever, skin rash, diarrhea, pancytopenia, and icterus). Skin biopsies demonstrated Grade II GVHD involvement. In both cases, sepsis and septic shock developed, with progression to multiorgan failure. Unfortunately, the 2 patients died, despite prompt, appropriate sepsis treatment and immunomodulatory therapy. TA-GVHD must be considered in the differential diagnosis of patients who present fever, pancytopenia, diarrhea, skin rash and icterus, and the transfusion history must be questioned.
authors.affiliation: Department of Pediatric Immunology, Uludag University, School of Medicine, Bursa, Turkey. sebnemkl@uludag.edu.tr
authors.forename: S
authors.initials: S
authors.lastname: Sebnem Kilic
authors.forename: S
authors.initials: S
authors.lastname: Kavurt
authors.forename: S
authors.initials: S
authors.lastname: Balaban Adim
mesh_terms: D042241:Early Diagnosis; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D005076:Exanthema; D017809:Fatal Outcome; D006086:Graft vs Host Disease / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology / Q000517:prevention & control; D006801:Humans; D007223:Infant; D008297:Male; D009102:Multiple Organ Failure; D012008:Recurrence; D012141:Respiratory Tract Infections; D016511:Severe Combined Immunodeficiency / Q000503:physiopathology / Q000628:therapy*; D012772:Shock, Septic; D012867:Skin / Q000276:immunology* / Q000473:pathology