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.
Department of Pediatric Immunology, University Medical Center, KC03.063.0, Lundlaan 6, 3584 EA Utrecht, The Netherlands. a.vanroyen@wkz.azu.nl
A
A
van Royen-Kerkhof
N M
NM
Wulffraat
S S M
SS
Kamphuis
R A
RA
Brooimans
R A
RA
de Weger
M G J
MG
Tilanus
E F
EF
van Leeuwen
G T
GT
Rijkers
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