Can diabetes insipidus be used as a marker for multisystemic and progressive disease in langerhans cell histiocytosis?

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease with an yearly incidence of nine cases per a million in children and 1-2 cases per a million adults. 68.6% of LCH presented with multisystem involvement. A 40-year-old woman who was admitted to endocrinology outpatient clinic with symptoms of polyuria, polydipsia and headache was diagnosed with diabetes insipidus (DI). Desmopressin treatment was initiated, but six months after therapy, re-evaluation revealed progression in hypophyseal mass. Thoracoscopic biopsy shows LCH with multisystemic involvement. She did not respond clinically to systemic chemotherapy and external radiotherapy and died due to pneumonia.LCH should be taken into consideration in patients diagnosed with DI. DI is almost always the hallmark of hypothalamic pituitary axis involvement and a sign of multisystemic involvement.