Langerhans Cell Histiocytosis with Central Diabetes Insipidus : A Case Report

중추성 요붕증이 동반된 랑게르한스 세포 조직구증 1예

  • Kim, Jin-Ho (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Moon, Jun-Sung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Mun, Sun-Jung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Lee, Ji-Eun (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Choi, Jae-Won (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Eun, Mi-Jung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Chun, Kyung-A (Department of Nuclear Medicine, College of Medicine, Yeungnam University) ;
  • Cho, Ihn-Ho (Department of Nuclear Medicine, College of Medicine, Yeungnam University) ;
  • Yoon, Ji-Sung (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Won, Kyu-Chang (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Lee, Kyung-Hee (Department of Internal Medicine, College of Medicine, Yeungnam University) ;
  • Shin, Duk-Seop (Department of Orthopedics, College of Medicine, Yeungnam University) ;
  • Lee, Hyoung-Woo (Department of Internal Medicine, College of Medicine, Yeungnam University)
  • 김진호 (영남대학교 의과대학 내과학교실) ;
  • 문준성 (영남대학교 의과대학 내과학교실) ;
  • 문선중 (영남대학교 의과대학 내과학교실) ;
  • 이지은 (영남대학교 의과대학 내과학교실) ;
  • 최재원 (영남대학교 의과대학 내과학교실) ;
  • 은미정 (영남대학교 의과대학 내과학교실) ;
  • 천경아 (영남대학교 의과대학 핵의학교실) ;
  • 조인호 (영남대학교 의과대학 핵의학교실) ;
  • 윤지성 (영남대학교 의과대학 내과학교실) ;
  • 원규장 (영남대학교 의과대학 내과학교실) ;
  • 이경희 (영남대학교 의과대학 내과학교실) ;
  • 신덕섭 (영남대학교 의과대학 정형외과학교실) ;
  • 이형우 (영남대학교 의과대학 내과학교실)
  • Published : 2005.12.30

Abstract

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

Keywords