Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease seen as part of multisystem Langerhans cell histiocytosis, or as an isolated form of the disease. The majority of cases of the latter are seen in adults and associated with cigarette smoking, which probably plays a central etiologic role. Although the mechanisms leading to the development of isolated PLCH are unc...

BACKGROUND Langerhans cell histiocytosis is a sporadic disease caused by an uncontrolled pathogenic clonal proliferation of dendritic cells that have Langerhans cell characteristics. New treatment protocols provided by the HISTSOC-LCH-III (NCT00276757) trial show an improvement in the survival of children with langerhans cell histiocytosis. CASE PRESENTATION We report a case of Langerhans cel...

Received 2/6/2007; revised 4/25/2007; accepted 4/27/2007. For correspondence or reprints contact: Wan-Yu Lin, M.D., Department of Medical Imaging, Taichung Veterans General Hospital. 160 Section 3, Taichung Harbor Road, Taichung 407, Taiwan. Tel: (886)4-23741349, Fax: (886)4-23741348, E-mail: wylin@ vghtc.gov.tw Langerhans’ cell histiocytosis (LCH) is a proliferative histiocytosis disorder. The...

Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations rangi...

Objective: Histiocytosis is a systemic disease that usually affects the central nervous system. The aim of this study is to discuss the neuroimaging characteristics of Langerhans cell histiocytosis (LCH), the most common of these diseases; and the non-Langerhans cells histiocytosis (NLCH), which includes entities such as hemophagocytic syndrome, Erdheim-Chester and Rosai-Dorfman diseases. Metho...

We present a case of 72-year-old male diagnosed with concurrent crystal-storing histiocytosis and nodal marginal zone lymphoma plasmacytic differentiation following finding new axillary lymphadenopathy during active surveillance for an early stage lung cancer.

Division and proliferation of dendritic cells (DCs) have been proposed to contribute to homeostasis and to prolonged antigen presentation. Whether abnormal proliferation of dendritic cells causes Langerhans cell histiocytosis (LCH) is a highly debated topic. Transgenic expression of simian virus 40 (SV40) T antigens in mature DCs allowed their transformation in vivo while maintaining their phen...

Solitary pulmonary Langerhans cell histiocytosis occurs predominantly in young adults, who are frequently heavy smokers. Besides the strong association to smoking, it differs from childhood Langerhans cell histiocytosis as well in that it is a polyclonal disorder, and the lesional Langerhans cells in this form of disease are reported to display mature markers. Thus, in this study we set out to ...

Adult pulmonary Langerhans’ cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers, with an incidence peak at 20–40 yrs of age. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease and is characterised by focal Langerhans’ cell granulomas infiltrating and destroying distal bronchioles. High-...

Langerhans cell histiocytosis (LCH) is a rare disease caused by the clonal accumulation of dendritic Langerhans cells, which is often accompanied by osteolytic lesions. It has been reported that osteoclast-like cells play a major role in the pathogenic bone destruction seen in patients with LCH and these cells are postulated to originate from the fusion of DCs. However, due to the lack of relia...