Acute antibody-mediated rejection in ABO-compatible pediatric liver transplant recipients: case series and review of the literature.
Acute AMR is well reported following ABO-incompatible LTx. However, it remains uncommon in ABO-compatible LTx. It typically presents with graft dysfunction ≤2 weeks post-LTx and is often associated with graft loss. We report the clinical presentation, treatment regimen, and outcome of six pediatric LTx recipients diagnosed with early acute AMR based on (i) clinical signs of graft dysfunction, (ii) histopathology indicative of acute injury ± C4d staining, and (iii) presence of HLA DSA. All patients developed elevated ALT and GGT ≤ 45 days post-LTx. All showed HLA class I (n=4) and/or II (n=6) DSA (peak MFI 6153-11 910). Four had de novo DSA, and two had preformed DSA. Five were initially diagnosed with ACR refractory to steroid therapy. Four exhibited resolution of graft dysfunction with AMR therapy. Two had refractory AMR-one was re-transplanted; the other was treated with eculizumab and showed improvement in graft function but later died due to a tracheostomy complication. Our case series suggests that AMR following ABO-compatible LTx may be under-diagnosed. The presentation can be variable, and treatment should be individualized. Eculizumab may be an option for refractory AMR. Ultimately, future multicenter studies are needed to better define diagnostic criteria, characterize optimal treatment, and assess long-term outcomes following liver AMR.
Pediatric Gastroenterology, Hepatology, and Nutrition, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Laura J
LJ
Wozniak
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Bita V
BV
Naini
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.|Immunogenetics Center, UCLA, Los Angeles, CA, USA.
Michelle J
MJ
Hickey
Medical Student Research Program, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Sarathi
S
Bhattacharyya
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.|Immunogenetics Center, UCLA, Los Angeles, CA, USA.
Elaine F
EF
Reed
Division of Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Ronald W
RW
Busuttil
Division of Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Douglas G
DG
Farmer
Pediatric Gastroenterology, Hepatology, and Nutrition, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Jorge H
JH
Vargas
Pediatric Gastroenterology, Hepatology, and Nutrition, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.|Division of Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Robert S
RS
Venick
Pediatric Gastroenterology, Hepatology, and Nutrition, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.|Division of Liver and Pancreas Transplantation, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Sue V
SV
McDiarmid
D000017:ABO Blood-Group System / Q000276:immunology*; D000906:Antibodies / Q000276:immunology*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection / Q000276:immunology*; D006085:Graft Survival; D006680:HLA Antigens / Q000276:immunology; D006650:Histocompatibility Testing; D006801:Humans; D007165:Immunosuppression Therapy; D007223:Infant; D007518:Isoantibodies / Q000276:immunology; D016031:Liver Transplantation; D008297:Male; D012189:Retrospective Studies; D014019:Tissue Donors; D016896:Treatment Outcome
D000017:ABO Blood-Group System; D000906:Antibodies; D061067:Antibodies, Monoclonal, Humanized; D006680:HLA Antigens; D007518:Isoantibodies; C481642:eculizumab
antibody-mediated rejection; donor-specific antibodies; liver transplantation