Histiocytosis, central diabetes insipidus, and pregnancy

Abstract

Dysfunction of the hypothalamus causes a significant polymorphism of the clinical symptomatology of diseases of the hypothalamic-pituitary system, associated with an excess or deficiency of pituitary hormones and the corresponding hyper- or hypofunction of the peripheral endocrine glands. In particular, a violation of the integrity of the hypothalamic-neurohypophysial tract, synthesis, transportation, and release of vasopressin leads to disorders of the water balance in the form of central diabetes insipidus. In 15-30% of all cases of diabetes insipidus, its primary (idiopathic) forms are detected. Other causes of neurogenic diabetes insipidus can be genetic abnormalities, infections, vascular diseases, pituitary surgery, tumors, traumatic brain injury, autoimmune and infiltrative processes. We present a case of a combination of central diabetes insipidus with histiocytosis from Langerhans cells (histiocytosis X) and pregnancy.

Histiocytosis X is a disease of the hematopoietic system, in which local or disseminated abnormal proliferation of macrophages occurs without signs of atypia with the formation of granulomas, eosinophilic infiltration in the internal organs, osteolytic foci. Since proliferating macrophages have structural and functional similarities with Langerhans cells, a new term was introduced in 1986 - histiocytosis from Langerhans cells.