Characteristics of long-term complications in Langerhans cell histiocytosis

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  • SHIODA Yoko
    Children’s Cancer Center, National Center for Child Health and Development
  • SAKAMOTO Kenichi
    Children’s Cancer Center, National Center for Child Health and Development Department of Pediatrics, Shinshu University School of Medicine
  • ONO Rintaro
    Department of Pediatrics, St. Luke’s International Hospital
  • FUJINO Hisanori
    Department of Pediatrics, Osaka Red Cross Hospital
  • KAWAHARA Yuta
    Department of Pediatrics, Jichi Medical University School of Medicine
  • KUDO Ko
    Department of Pediatrics, Hirosaki University School of Medicine
  • SUENOBU Soichi
    Oita University Faculty of Medicine
  • DOI Takehiko
    Department of Pediatrics, Hiroshima University Graduate School of Biomedical and Health Sciences
  • SATO Aki
    Department of Hematology and Oncology, The Institute of Medical Science Research Hospital, The University of Tokyo
  • KUDO Kazuko
    Department of Pediatrics, Fujita Health University School of Medicine
  • MORIMOTO Akira
    Department of Pediatrics, Jichi Medical University School of Medicine

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Other Title
  • ランゲルハンス細胞組織球症における晩期合併症の特徴
  • ランゲルハンス サイボウ ソシキキュウショウ ニ オケル バンキ ガッペイショウ ノ トクチョウ

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Description

<p>About 100 cases of Langerhans cell histiocytosis (LCH) occur annually in Japan. It predominantly occurs in infants, presenting as multisystem disease or multifocal bone involvement. However, LCH can also occur in adults aged 20 to 40. Single-system skin involvement is rare, with most cases presenting with multisystem disease, including bone lesions, which respond to chemotherapy. In adults, lung lesions that improve with smoking cessation are well-known, although multisystem disease is more common and requires aggressive therapeutic intervention similar to that in children. In some infant cases, progression of liver, spleen, and bone marrow lesions can be difficult to control and can become severe. However, targeted molecular therapies are now available as a lifesaving option. More than 30% of cases of multisystem LCH recur at least once, often leading to long-term complications. In particular, the emergence of central diabetes insipidus, anterior pituitary dysfunction, and central nervous system neurodegenerative disorders several years after the diagnosis of LCH is a unique feature not observed in other diseases. New therapeutic strategies are needed to counter these problems.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 65 (9), 1216-1226, 2024

    The Japanese Society of Hematology

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