Langerhans cell histiocytosis includes a broad spectrum of clinical diseases resulted from proliferation and tissue infiltration of differentiated langerhans cells. This term includes Letterer-siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma and congenital reticulohistiocytosis that are more common in children than adults. This entity has recently been classified into restr...

Abstract Background. Langerhans cell histiocytosis is a rare reactive proliferative disorder marked by excess proliferation and accumulation of mononuclear phagocytes in tissues organs. Usually, organs systems where cells are normally found involved. Lesions may be limited to one system single- or multisite, many The etiology not fully known. According the hypotheses, immune dysfunction due exu...

Langerhans cell histiocytosis is characterized by abnormal accumulations of large mononuclear cells forming granulomas in various organs mainly in the lung, bone, or skin. Adult pulmonary Langerhans cell histiocytosis is rare and almost always associated with cigarette smoking; combination with lung and bone simultaneous involvement is even rare. We present a 41 years old male smoker who was di...

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...

Vertebral Langerhans cell histiocytosis, formerly called Histiocytosis X, is rarely seen in adults: a systematic non-quantitative review of the literature yielded only 27 cases. Vertebra plana is often associated in children, but this is not a feature in the adult population. The authors report the case of a 29-year-old woman with a two-month history of lumbar pain. Osteolysis of the right pedi...

UNLABELLED Rosai-Dorfman Syndrome (RDD) is a rare, benign, self-limiting disorder which is characterized by the non-malignant proliferation of distinctive histiocytic cell within lymphatic system. RDD has been described as a dynamic entity in the spectrum of histiocytosis with non-Langerhans cell histiocytosis at one end and Langerhans cell histiocytosis at the other. The exact etiology of this...

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...

Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus...

Five cases of malignant histiocytosis of the intestine and one case of true histiocytic lymphoma were studied using immunohistological techniques. In paraffin sections tumour cells in all cases were shown to contain alpha-1-antitrypsin and to express the leucocyte common antigen. Four of the five cases of malignant histiocytosis of the intestine and the case of histiocytic lymphoma expressed th...

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 ...