Langerhans Cell Histiocytosis: A Complicated Case Report and Literature Review

Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare disease of unknown etiology, characterized by monoclonal proliferation and organ infiltration of Langerhans cells. The clinical manifestations are various, and may involve single or multiple organs. A case of LCH is introduced and the diagnosis and treatment are discussed. Case presentation: A 23-year-old woman presented with a huge mass in the left lower abdomen. Computed tomography (CT) of the lung revealed multiple diffuse nodules of different sizes in the right lung. The left thorax was collapsed, the left pleura was thickened and adhered, and a small, encapsulated effusion was observed in the thoracic cavity. Enhanced CT of the whole abdomen showed a huge cystic low-density focus on the left lower abdomen, multiple nodules in the spleen, and multiple osteolytic bone destruction in the spine, thorax, and pelvis. The possibility of LCH was considered based on the presence of diffuse reticulum nodules in the lungs observed in CT imaging. The patient was performed ultrasound-guided abdominal puncture, drainage, and chemotherapy with "vinorelbine and cisplatin". Follow-up is ongoing. Conclusion: LCH involving the serosal membrane is rare. The final diagnosis requires histopathological examination of the involved tissue. But in most cases, it’s enough to diagnose through the typical imaging changes in the lung, as observed by high-resolution computed tomography (HRCT), combined with certain clinical observations. Currently, there are no clearly established treatment guidelines for LCH. At present, systemic chemotherapy is the main treatment method that has been shown to improve patient’s prognosis.