Spontaneous Gonadotrophin Deficiency Recovery in an Adult Patient with Langerhans Cell Histiocytosis (LCH)
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- Makras, P., Papadogias, D., Kontogeorgos, G. et al. Pituitary (2005) 8: 169. doi:10.1007/s11102-005-4537-z
Langerhans cell histocytosis (LCH) is a rare disease which exhibits a particular predilection for pituitary involvement leading to diabetes insipidus (DI) and other anterior pituitary hormonal deficiencies that are usually permanent and unresponsive to treatment. We report a 35 year old woman with a 10 year history of multisystemic LCH who developed DI, mild hyperprolactinemia, gonadotrophin and partial growth hormone deficiency following a normal delivery that was accompanied with infundibular thickening on pituitary magnetic resonance imaging (MRI). Following several courses of glucocorticoid administration, that were not associated with any substantial improvement, the patient was started on estrogen replacement therapy and cabergoline. After a three year period free of further relapses she developed irregular uterine bleeding. Following estrogen and cabergoline discontinuation she resumed normal menstruation while a repeated MRI of the pituitary showed an almost normal infundibulum. Endocrine investigation revealed normal gonadotrophin axis and prolactin levels, while the patient continues to menstruate, every 30–40 days, ten months after the resumption of her menstrual cycle. This case demonstrates for the first time that LCH induced pituitary deficiencies can run a variable clinical course and even spontaneously recover.
Key wordsLCHhypopituitarismgonadotrophin axis
Langerhans cell histocytosis
magnetic resonance imaging;
transfer factor for carbon monoxide;
computerized tomography; BMI, body mass index.
body mass index.