Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of cellular blood component transfusion that produces a graftversus-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.

Key words: Transfusion-associated graft-versus-host disease. Severe combined immunodeficiency. Irradiation. Skin biopsy.