title: Rapidly progressing fatal adult multi-organ Langerhans cell histiocytosis complicated with fatty liver disease.
abstract: Langerhans cell histiocytosis (LCH) is a clonal neoplasm that shows diverse clinical manifestations and courses of disease progression. The etiology and pathophysiology of LCH remain uncertain. We describe the clinical course of a 23-year-old Japanese woman with multi-system LCH, who showed rapid progression after steroid reduction and developed multi-organ failure. Liver biopsy showed LCH infiltration with fatty degeneration. She was treated with cytarabine, vincristine, and prednisolone according to the Japan LCH study group 02 protocol, without any clinical improvement. Low expression of Ki67 and bcl-2 failed to explain the rapid clinical course. Panhypopituitarism and hypothalamic dysfunction may have caused nonalcoholic fatty liver disease and liver failure. This case indicates that some multi-system LCH patients with hypopituitarism and hypothalamic dysfunction show very rapid progression and are difficult to treat.
authors.affiliation: Departments of Hematology, Jichi Medical University, Shimotsuke, Tochigi, Japan. mitsushak-tky@umin.ac.jp
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Yuasa
authors.forename: Shinichirou
authors.initials: S
authors.lastname: Fujiwara
authors.forename: Iekuni
authors.initials: I
authors.lastname: Oh
authors.forename: Takehiko
authors.initials: T
authors.lastname: Yamaguchi
authors.forename: Noriyoshi
authors.initials: N
authors.lastname: Fukushima
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
authors.forename: Keiya
authors.initials: K
authors.lastname: Ozawa
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D018450:Disease Progression; D017809:Fatal Outcome; D005234:Fatty Liver / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D015870:Gene Expression; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007018:Hypopituitarism / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D007031:Hypothalamus / Q000473:pathology; D019394:Ki-67 Antigen / Q000235:genetics; D009102:Multiple Organ Failure / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D065626:Non-alcoholic Fatty Liver Disease; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics; D055815:Young Adult
chemical_list: D019394:Ki-67 Antigen; D019253:Proto-Oncogene Proteins c-bcl-2