Журнал клинических случаев

Langerhans Cell Histiocytosis with Multisystem Involvement in a Young Woman: A Case Report and Literature Review

Abstract

Sheniz Yuzeir, Hinko Varbanov, Tsvetelina Yordanova and Ilina Micheva

Langerhans Cell Histiocytosis (LCH), also known as Histiocytosis X (HX), is a group of hyperplastic cellular diseases of unknown causes. LCH could affect bones, lungs, central nervous system, liver, thymus, skin, and also lymph nodes. The diagnosis of LCH is difficult to enforce and rarely found in adults, with just about 5 cases per million per year. The present study reports the case of a young woman with LCH with multisystem involvement, including that of the bone, orbit, pulmonary system and central nervous system. The patient received chemotherapy for 6 months and exhibited rapid improvement in the involved systems. The last PET/CT showed metabolic activity in the right iliac bone. One year after completion of the therapy, the patient returned to the hospital showing deteriorating health. The clinical case is interesting not only because of the registered clinical, morphological, and imaging data of histiocytosis but also because of the unclear prognostic and diagnostic importance of this phenomenon.