Clinical and prognostic features of Langerhans cell histiocytosis in adults.

ABSTRACT: Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal expansion of CD1a⁺ CD207⁺ myeloid dendritic cells. The features of LCH are mainly described in children and remain poorly defined in adults; therefore, we conducted a nationwide survey to collect clinical data from 148 adult patients with LCH. The median age at diagnosis was 46.5 (range: 20-87) years with male predominance (60.8%). Among the 86 patients with detailed treatment information, 40 (46.5%) had single system LCH, whereas 46 (53.5%) had multisystem LCH. Moreover, 19 patients (22.1%) had an additional malignancy. BRAF V600E in plasma cell-free DNA was associated with a low overall survival (OS) rate and the risk of the pituitary gland and central nervous system involvement. At a medi

SUBMITTER: Sato A 

PROVIDER: S-EPMC10475785 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature