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Optimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report

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Abstract

Liver cirrhosis due to secondary sclerosing cholangitis caused by Langerhans cell histiocytosis (LCH) has a poor prognosis, and liver transplantation is the definitive treatment. However, the optimal timing has not been established. We report a 2-year-old girl with LCH-related liver cirrhosis who successfully underwent liver transplantation before progressing to severe liver dysfunction. Physical examination revealed a tumor on her palate. Biopsy was performed, and a diagnosis of LCH was established, together with hepatomegaly, splenomegaly, and rashes. Percutaneous liver biopsy before treatment revealed extreme fibrosis and absence of LCH cells. After beginning chemotherapy, she experienced several delays in treatment and dose reductions because of unacceptable bone marrow suppression, worsening liver dysfunction, and cholangitis. However, tumor shrinkage was observed in both magnetic resonance imaging and BRAF V600E mutant allele titers in her plasma. Given the good treatment response, liver transplantation was conducted. The postoperative course was uneventful, and chemotherapy was resumed 34 days after liver transplantation. Subsequent maintenance treatment was completed with no severe adverse effects. To prevent perioperative complications due to exacerbation of liver dysfunction and possible discontinuation of chemotherapy, liver transplantation should be considered before development of end-stage liver failure, provided that the original disease is well controlled.

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Authors and Affiliations

  1. Department of Hematology/Oncology, Saitama Children’s Medical Center, 1-2, Shin-Toshin, Saitama, 330-8777, Japan

    Mai Watakabe, Kohei Fukuoka & Katsuyoshi Koh

  2. Department of Transplant Surgery, Saitama Children’s Medical Center, Saitama, Japan

    Yoshiyuki Ihara, Megumi Tamura & Koichi Mizuta

  3. Division of Gastroenterology and Hepatology, Saitama Children’s Medical Center, Saitama, Japan

    Tomoko Hara & Itaru Iwama

  4. Department of Pediatrics, Hirosaki University Graduate School of Medicine, Hirosaki, Japan

    Ko Kudo

  5. Department of Pathology, Saitama Children’s Medical Center, Saitama, Japan

    Kayoko Ichimura & Atsuko Nakazawa

  6. Department of Radiology, Saitama Children’s Medical Center, Saitama, Japan

    Yutaka Tanami

  7. Department of Pediatric Surgery, Saitama Children’s Medical Center, Saitama, Japan

    Hiroshi Kawashima

  8. Department of Clinical Research, Saitama Children’s Medical Center, Saitama, Japan

    Atsuko Nakazawa

Authors
  1. Mai Watakabe
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  2. Kohei Fukuoka
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  3. Yoshiyuki Ihara
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  4. Tomoko Hara
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  5. Ko Kudo
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  6. Megumi Tamura
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  7. Kayoko Ichimura
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  8. Yutaka Tanami
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  9. Hiroshi Kawashima
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  10. Itaru Iwama
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  11. Atsuko Nakazawa
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  12. Koichi Mizuta
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  13. Katsuyoshi Koh
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Correspondence to Kohei Fukuoka.

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Watakabe, M., Fukuoka, K., Ihara, Y. et al. Optimal timing of liver transplantation for liver cirrhosis caused by sclerosing cholangitis in a patient with Langerhans cell histiocytosis: a case report. Int J Hematol 117, 759–764 (2023). https://doi.org/10.1007/s12185-022-03500-y

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  • DOI: https://doi.org/10.1007/s12185-022-03500-y

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