ABSTRACT
Objective To observe the clinical characteristics, treatment and prognosis of adult patients with langerhans cell histiocytosis (LCH). Methods The clinical data of 21 adult patients with LCH≥18 years old from March 2010 to March 2017 in the Second Affiliated Hospital of Southeast University and the First Affiliated Hospital with Nanjing Medical University were retrospectively analyzed, and the clinical manifestations, laboratory tests, treatments and prognosis were observed. Results A total of 21 patients included 14 male cases and 7 female cases. The median age was 43 years old (22-62 years old). There were 4 patients with single system and single lesion, 5 patients with single system and multi-lesion (Hand-Schuller-Christian) and 12 patients with multisystem and multi-lesion (Letter-Siwe disease). Risk-organ involvement was observed in 7 cases (3 lung cases, 2 bone marrow cases, 1 liver case and 1 spleen case ). The median overall survival time of all patients with LCH was 36 months (1-89 months), including 9 patients with single-system disease and 5 patients with multisystem disease without recurrence and unstable condition (survival time: 4-89 mouths). Among 7 patients with multisystem disease with high-risk organ involvement, 3 survived with no recurrence and 4 died from disease progression. Conclusions The incidence of adult LCH featured by the involvement of more multisystem and multi-lesion in clinic is low, and male cases are in the majority. Patients with multisystem disease and risk organ involvement have poor response to current therapy, and new treatments need to be explored.