Clinical case of Langerhans cell histiocytosis in endocrinological practice

T.Yu. Demidova, Ye.Yu. Gritskevich, Ye.V. Volkova

Pirogov Russian National Research Medical University, Moscow

The article describes a clinical case of a rare disease — Langerhans cells histiocytosis, formerly histiocytosis X, which etiology is unknown. The main mechanisms of disease progression are a clonal proliferation of pathological Langerhans cells and abnormal regulation of immune system cells interaction in lesions. Cell infiltration has polymorphic nature and contains lymphocytes, eosinophils, monocytes, macrophages alongside pathological Langerhans cells. Langerhans cells histiocytosis pathogenesis combines patterns of neoplasm and reactive process. The peak incidence is in early childhood. The disease clinical picture varies from localized forms with the benign course and spontaneous recovery to disseminated forms with a rapidly progressive course and fatal outcome. Pathological process can involve any organs and systems in different combinations: skeleton, skin, lymph nodes, outer ear, liver, spleen, bone marrow, lungs, endocrine glands, central nervous system, gastrointes tinal tract, thymus. The peculiarity of this clinical case is the disease manifestation in adulthood with endocrinopathy: development of diabetes insipidus, hyperprolactinemia, amenorrhea.

Keywords: Langerhans cells histiocytosis, diabetes insipidus, hyperprolactinemia, autoimmune thyroiditis, amenorrhea.

For citation: Demidova T.Yu., Gritskevich Ye.Yu., Volkova Ye.V. Clinical case of Langerhans cell histiocytosis in endocrinological practice.
RMJ. 2019;12:25–28.