The histiocytoses are a group of proliferative disorders of themonocyte-macrophage lineage that are neoplastic or reactive innature. Based on immunophenotyping and electron microscopy,two main groups have been recognized namely 1) Langerhanscell histiocytosis (LCH) and 2) non- Langerhans cell histiocytosis(non-LCH). In this study, a fairly rare disease of the non-LCHgroup, generalized eruptive ...

Langerhans cell histiocytosis following T-ALL: clonally related neoplasms with persistent expression of constitutively active NOTCH1. Am J Hematol. 2008;83(2): 116-121. 7. Coury F, Annels N, Rivollier A, et al. Langerhans cell histiocytosis reveals a new IL-17A-dependent pathway of dendritic cell fusion. Nat Med. 2008;14(1):81-87. 8. Allen CE, McClain KL. Interleukin-17A is not expressed by CD2...

BACKGROUND Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, ...

langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection is very rare. the purpose of this report is to describe a un...

Block 6 Level 9, Dermatology Unit, Singapore General Hospital, Outram Road, 169608, Singapore Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause characterized by the accumulation of Langerhan cells in various tissues. Typically a disorder of childhood, features of this disease have been well documented in children but remain ambiguous and poorly documen...

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-...

B cell non-Hodgkin lymphoma of the follicular subtype (grade 3/3) affecting the nasopharynx and breast, and containing foci of Langerhans cell histiocytosis, was diagnosed in a 56 year old white woman who was a longstanding heavy smoker. Four years before this she had developed stage 1a mixed cellularity Hodgkin lymphoma affecting the right inguinal region, which was treated by irradiation and ...

Langerhans cell histiocytosis can involve single or multiple organ/tissue systems and may go undiagnosed for years until it enters the clinician's differential diagnosis framework. We report on a young patient who initially presented with diabetes insipidus and subsequently with pyrexia of unknown origin. She progressed from single system Langerhans cell histiocytosis to multisystem involvement...