Multisystemic Langerhans cell histiocytosis in an adult

The etiology of Langerhans cell histiocytosis (LCH), a mix between immune dysregulation, inflammation, and malignancy, remains unclear.1, 2, 3, 4 In half of the patients, an oncogenic BRAF mutation is found.3 Because of the diversity of symptoms, the diagnosis of LCH, as defined by the Histiocyte Society, is often made with considerable delay. Multisystemic LCH, affecting 2 or more organ systems and “risk organs,” like the hematopoietic system, the spleen, liver, and central nervous system, implies a worse prognosis.1, 3, 4, 5, 6 To raise the awareness of multisystemic LCH, the case of an elderly patient is presented and a new therapeutic scheme with pioglitazone (peroxisome proliferator–activated receptor-γ [PPAR-γ] agonist), etoricoxib (COX-2 inhibitor), and trofosfamide (alkylating medium) is described.