Yeungnam Univ J Med.  2005 Dec;22(2):259-265.

Langerhans Cell Histiocytosis with Central Diabetes Insipidus: A Case Report

• Kim JH
• Moon JS
• Mun SJ
• Lee JE
• Choi JW
• Eun MJ
• Chun KA
• Cho IH
• Yoon JS
• Won KC
• Lee KH
• Shin DS
• Lee HW

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.

Keyword

Diabetes insipidus; Langerhans cell histiocytosis

MeSH Terms

Adult
Biopsy
Brain
Deamino Arginine Vasopressin
Diabetes Insipidus
Diabetes Insipidus, Neurogenic*
Diagnosis
Drug Therapy
Etoposide
Female
Hip
Histiocytes
Histiocytosis, Langerhans-Cell*
Humans
Lymphocytes
Magnetic Resonance Imaging
Osmotic Pressure
Pituitary Gland
Polydipsia
Polyuria
Thirst
Vasopressins
Water Deprivation
Deamino Arginine Vasopressin
Etoposide
Vasopressins