Langerhans cell histiocytosis is a disease which frustrates both clinician and scientist.
Its aetiology is unknown, its pathogenesis is ill understood and the clinical course is unpredictable.
Historically, the different nomenclatures reflecting the first clinical descriptions by Hand (1893,1921), Schuller (1915) and Chrisiian (1920), and subsequently by Letterer (1924) and Siwe (1933), led to confusion only partially resolved by Lichtenstein (1953) who recognised that the disease in each of these clinical syndromes were components of a spectrum of disease involving the histiocyte.
He proposed his unifying concept of Histiocytosis X- « X » being the unknown aetiological factor.
Mots-clés Pascal : Histiocytose langerhansienne, Diagnostic, Epidémiologie, Homme, Hémopathie
Mots-clés Pascal anglais : Langerhans cell histiocytosis, Diagnosis, Epidemiology, Human, Hemopathy
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 94-0647002
Code Inist : 002B19D. Création : 09/06/1995.