Langerhans cell histiocytosis
Pronunciation: LANG-er-hahns
Also written as: LCH — Langerhans cell histiocytosis
A rare disorder involving abnormal proliferation and accumulation of dendritic cells, presenting with varied manifestations in pediatric patients.
Full Definition
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation and tissue infiltration of abnormal dendritic cells resembling Langerhans cells. In pediatric patients, LCH can present as single-system disease (commonly affecting bone or skin) or multisystem disease involving organs such as the liver, spleen, bone marrow, or central nervous system. The condition may be associated with BRAF V600E mutations. Clinical manifestations are highly variable, ranging from isolated bone lesions to life-threatening multiorgan dysfunction. Diagnosis requires histologic confirmation showing characteristic cells with coffee-bean nuclei, positive CD1a and CD68 staining, and Birbeck granules on electron microscopy.
Usage
Usage note: Commonly abbreviated as LCH; formerly known as histiocytosis X.
In Context
- "The child's lytic skull lesions and characteristic histologic findings confirmed a diagnosis of Langerhans cell histiocytosis." — Oncology consultation note