Langerhans Cell Histiocytosis - A Rare Case Diagnosed Cytologically

LCH is a rare idiopathic disorder characterized by pathogenic clonal proliferation of bone marrow derived langerhans cells which are immature dendritic cells characterized by presence of Birbeck granules. The annual incidence of LCH is nearly 5.4cases per one million people with male predominance. LCH is primarily a pediatric disease with peak age ranging between 1-4 yrs commonly involving bone. It manifests as single system unifocal, multifocal or multisystem disease. The purpose of this case report is to describe a case of 10 month old male baby with unifocal bone involvement and discuss the clinical, radiological and characteristic cytological features that lead to the diagnosis of LCH.

A Case of Erdheim-Chester Disease with periodic fever and knee pain / 대한내과학회지

ECD is a rare, non-Langerhans form of histiocytosis. The first case was reported in 1930 by Chester and the term ECD was first described by Jaffe in 1972. Many authors have suggested that ECD is a part of the spectrum of Langerhans cell histiocytosis(LCH), whereas others believe ECD as a distinct lipoidosis because of the symmetric distribution of the long bone lesions on the roentgenogram and the lack of Langerhans granules and S-100 antibody staining in the foamy histiocytes. We experienced a case of Erdheim-Chester disease (ECD) with periodic night fever, myalgia and both knee pain, the first case reported in Korea.