内科: オープンアクセス
オープンアクセス

概要

Langerhans Cell Histiocytosis: A Rare Cause of Central Diabetes Insipidus

Weerachai Srivanichakorn, Jantima Tanboon and Apiradee Sriwijitkamol

Objective: To report adult patients with Langerhans cell histiocytosis who presented with a recent onset of polyuria, secondary amenorrhea and galactorrhea.
Methods: We report the clinical presentations, laboratory test results, imaging findings, histological findings and clinical courses of two cases of adult Langerhans cell histiocytosis.
Results: Our evaluation revealed the presence of central diabetes insipidus. Magnetic resonance imaging of the pituitary showed a thickening of the pituitary stalk. The skeleton survey, chest radiography and biochemistry revealed an osteolytic lesion at the frontal bone only in the first case; the other case was within normal limits. A biopsy was performed at the frontal bone in the first case and at the pituitary stalk in the other case. The pathologic examination confirmed the diagnosis of Langerhans cell histiocytosis. Replacement therapy with an intranasal administration of desmopressin can resolve symptoms. The stalk lesion decreased in size without specific treatment.
Conclusions: We reported two cases of adult Langerhans cell histiocytosis. These patients presented with central diabetes insipidus and galactorrhea. Both MRI findings revealed a unifocal infiltration of the infundibulum. The pathologic examination confirmed the diagnosis of Langerhans cell histiocytosis. Replacement therapy with an intranasal administration of desmopressin helped resolve symptoms. The stalk lesion decreased in size without specific treatment. Long-term follow up with proper supplementation to correct hormonal deficiencies may be the proper management in patients with isolated sellar or bone involvement that frequently experience a slower progression of the disease.