Nonlethal transfusion associated graft-versus-host disease in a severe combined immunodeficient patient |
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Authors: | van Royen-Kerkhof A Wulffraat N M Kamphuis S S M Brooimans R A de Weger R A Tilanus M G J van Leeuwen E F Rijkers G T |
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Affiliation: | Department of Pediatric Immunology, University Medical Center, KC03.063.0, Lundlaan 6, 3584 EA Utrecht, The Netherlands. a.vanroyen@wkz.azu.nl |
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Abstract: | An X-linked severe combined immunodeficient (SCID) patient received a nonirradiated erythrocyte transfusion and developed transfusion-associated graft-versus-host disease (TAGVHD), which was controllable with high-dose corticosteroids. Haplo-identical SCT was performed, after a myeloablative conditioning regimen. At day +26, he developed GVHD. Chimerism studies revealed DNA of the erythrocyte transfusion donor (ETD) and recipient only. Because of early nonengraftment and the presence of alloreactive T cells of ETD origin, the patient was treated with an immunosuppressive conditioning regimen followed by a second SCT from the same donor. While tapering immunosuppression, he again developed mild GVHD, and DNA of ETD and bone marrow donor origin were both present. On cyclosporin, the ETD-DNA signal finally disappeared. High-resolution HLA typing revealed haplo-identity between BMD, ETD and the patient, which might have contributed to the relative mild course of the TAGVHD. |
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