Nonlethal transfusion associated graft-versus-host disease in a severe combined immunodeficient patient.
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.
van Royen-Kerkhof
A
A
Department of Pediatric Immunology, University Medical Center, KC03.063.0, Lundlaan 6, 3584 EA Utrecht, The Netherlands. a.vanroyen@wkz.azu.nl
Wulffraat
N M
NM
Kamphuis
S S M
SS
Brooimans
R A
RA
de Weger
R A
RA
Tilanus
M G J
MG
van Leeuwen
E F
EF
Rijkers
G T
GT
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D006086:Graft vs Host Disease / Q000188:drug therapy / Q000209:etiology*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000628:therapy*; D018183:Transplantation Chimera / Q000235:genetics; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones