title: Antibody-mediated rejection after ABO-incompatible pediatric living donor liver transplantation for propionic acidemia: A case report.
abstract: We herein present the case of a four-yr-old boy with PA who developed AMR after ABO-incompatible LDLT despite undergoing B cell desensitization using rituximab. Although the CD19+ lymphocyte count decreased to 0.1% nine days after the administration of rituximab, he developed a high fever which was accompanied by arthralgia due to a streptococcal infection 13 days after rituximab prophylaxis. After the clearance of the infection, he underwent ABO-incompatible LDLT 36 days after the administration of rituximab. The CD19+ lymphocyte count just prior to LDLT was 1.2%. He developed AMR five days after LDLT, and the antidonor-type IgM and IgG antibody titers increased to 1:1024 and 1:1024, respectively. He was treated by plasma exchange, IVIG, steroid pulse therapy, and rituximab re-administration; however, his liver dysfunction continued. Despite intensive treatment, he died due to complicated abdominal hernia, acute renal failure, and ARDS. This case suggests that a streptococcal infection may induce the activation of innate immune responses; thus, additional desensitization therapy should be considered prior to ABO-incompatible LDLT if B cell reactivation is suspected.
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Honda
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.affiliation: Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Rieko
authors.initials: R
authors.lastname: Sakamoto
authors.affiliation: Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Shirou
authors.initials: S
authors.lastname: Matsumoto
authors.affiliation: Department of Pediatric Surgery, Kumamoto City Hospital, Kumamoto, Japan.
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Irie
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Koushi
authors.initials: K
authors.lastname: Uchida
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Keita
authors.initials: K
authors.lastname: Shimata
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Kawabata
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Kaori
authors.initials: K
authors.lastname: Isono
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Shintaro
authors.initials: S
authors.lastname: Hayashida
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Yamamoto
authors.affiliation: Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Fumio
authors.initials: F
authors.lastname: Endo
authors.affiliation: Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Inomata
mesh_terms: D000017:ABO Blood-Group System / Q000276:immunology*; D001787:Blood Group Incompatibility / Q000276:immunology*; D002675:Child, Preschool; D017809:Fatal Outcome; D006084:Graft Rejection / Q000175:diagnosis / Q000276:immunology*; D006801:Humans; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D056693:Propionic Acidemia / Q000601:surgery*
chemical_list: D000017:ABO Blood-Group System
keywords: antibody-mediated rejection; propionic acidemia; rituximab; streptococcal infection