Alteraciones endocrinas en las histiocitosis de la región hipotálamo hipofisaria / Endocrine changes in histiocytosis of the hypothalamic-pituitary axis

Las histiocitosis son cuadros caracterizados por la proliferación de células del sistema mononuclear fagocítico. Incluyen la histiocitosis de células de Langerhans (HCL) y las histiocitosis de células no Langerhans (entre ellas la enfermedad de Chester-Erdheim [ECE]). Aunque la diabetes insípida (DI) es la alteración hipotálamo hipofisaria (HH) más frecuente, están menos estudiados los déficits hipofisarios anteriores. Se analiza la frecuencia y la progresión de los déficits hormonales hipofisarios y los hallazgos radiológicos de 9 pacientes (7 HCL y 2 ECE) con afectación de la región HH. El 89% de los pacientes presentaba DI (62% al diagnóstico) y el 78%, uno o más déficits anteriores (71% al diagnóstico). Dado que la afectación HH es relativamente frecuente en pacientes con diagnóstico de histiocitosis y que los déficits hormonales pueden estar presentes al diagnóstico o aparecer de forma paulatina durante el curso de la enfermedad, es recomendable monitorizar de manera regular a este tipo de pacientes

Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended

Endocrine changes in histiocytosis of the hypothalamic-pituitary axis.

Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended.