1. Nonlethal transfusion associated graft-versus-host disease in a severe combined immunodeficient patient.
- Author
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van Royen-Kerkhof A, Wulffraat NM, Kamphuis SS, Brooimans RA, de Weger RA, Tilanus MG, van Leeuwen EF, and Rijkers GT
- Subjects
- Adrenal Cortex Hormones therapeutic use, Graft vs Host Disease drug therapy, Hematopoietic Stem Cell Transplantation, Humans, Infant, Male, Severe Combined Immunodeficiency complications, Transplantation Chimera genetics, Treatment Outcome, Erythrocyte Transfusion adverse effects, Graft vs Host Disease etiology, Severe Combined Immunodeficiency therapy
- 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.
- Published
- 2003
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