Volume 20, Issue 6 pp. 840-845
Case Report

Antibody-mediated rejection after ABO-incompatible pediatric living donor liver transplantation for propionic acidemia: A case report

Masaki Honda

Corresponding Author

Masaki Honda

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

Masaki Honda, Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo-ku, Kumamoto 860-8556, Japan

Tel.: +81 96 373 5616

Fax: +81 96 373 5616

E-mail: [email protected]

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Seisuke Sakamoto

Seisuke Sakamoto

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Rieko Sakamoto

Rieko Sakamoto

Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Shirou Matsumoto

Shirou Matsumoto

Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Tomoaki Irie

Tomoaki Irie

Department of Pediatric Surgery, Kumamoto City Hospital, Kumamoto, Japan

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Koushi Uchida

Koushi Uchida

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Keita Shimata

Keita Shimata

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Seiichi Kawabata

Seiichi Kawabata

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Kaori Isono

Kaori Isono

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Shintaro Hayashida

Shintaro Hayashida

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Hidekazu Yamamoto

Hidekazu Yamamoto

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Fumio Endo

Fumio Endo

Department of Pediatrics, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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Yukihiro Inomata

Yukihiro Inomata

Department of Transplantation and Pediatric Surgery, Postgraduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan

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First published: 20 July 2016
Citations: 8

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.

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