Unifocal Langerhans cell histiocytosis localised to the pituitary gland or hypothalamus can produce significant endocrine consequences in adults. When the disease results in central diabetes insipidus alongside anterior pituitary dysfunction, a specific management approach applies.
Adult with unifocal LCH of the pituitary/hypothalamus presenting with central diabetes insipidus and anterior pituitary dysfunction — recognised endocrine sequelae of this localisation.
Management in this setting centres on hormone replacement directed at the pituitary/hypothalamic endocrinopathy. The complete structured regimen is available in the full protocol.
For unifocal LCH of pituitary/hypothalamus resulting in DI and anterior pituitary dysfunction, hormone replacement should be undertaken.
DOI: 10.1182/blood.2021014343
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