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[Characteristics of long-term complications in Langerhans cell histiocytosis].
Shioda, Yoko; Sakamoto, Kenichi; Ono, Rintaro; Fujino, Hisanori; Kawahara, Yuta; Kudo, Ko; Suenobu, Soichi; Doi, Takehiko; Sato, Aki; Kudo, Kazuko; Morimoto, Akira.
Afiliação
  • Shioda Y; Children's Cancer Center, National Center for Child Health and Development.
  • Sakamoto K; Children's Cancer Center, National Center for Child Health and Development.
  • Ono R; Department of Pediatrics, Shinshu University School of Medicine.
  • Fujino H; Department of Pediatrics, St. Luke's International Hospital.
  • Kawahara Y; Department of Pediatrics, Osaka Red Cross Hospital.
  • Kudo K; Department of Pediatrics, Jichi Medical University School of Medicine.
  • Suenobu S; Department of Pediatrics, Hirosaki University School of Medicine.
  • Doi T; Oita University Faculty of Medicine.
  • Sato A; Department of Pediatrics, Hiroshima University Graduate School of Biomedical and Health Sciences.
  • Kudo K; Department of Hematology and Oncology, The Institute of Medical Science Research Hospital, The University of Tokyo.
  • Morimoto A; Department of Pediatrics, Fujita Health University School of Medicine.
Rinsho Ketsueki ; 65(9): 1216-1226, 2024.
Article em Ja | MEDLINE | ID: mdl-39358280
ABSTRACT
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Histiocitose de Células de Langerhans Limite: Humans Idioma: Ja Revista: Rinsho Ketsueki Ano de publicação: 2024 Tipo de documento: Article País de publicação: Japão
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Histiocitose de Células de Langerhans Limite: Humans Idioma: Ja Revista: Rinsho Ketsueki Ano de publicação: 2024 Tipo de documento: Article País de publicação: Japão