Idiopathic myelofibrosis physiopathology: from mice to human Volume 12, issue 5, Septembre-Octobre 2006


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INSERM U790, Institut Gustave-Roussy, Pavillon de recherche 1, 39 rue Camille-Desmoulins, 94805 Villejuif Cedex, France, Laboratoire d’hématologie, Hôpital Henri-Mondor, Créteil, France

Agnogenic myeloid metaplasia or idiopathic myelofibrosis become the more severe “classical” myeloproliferative disorder since Gleevec ® have been used in clinical practice to treat chronic myeloid leukemia patients. Interestingly, this disorder have two mains characteristics: a myeloproliferation and a stromal secondary reaction that could be responsible from a part of the disease morbidity. Molecular mechanisms leading to the development of this disorder seems to be better understood because of the works performed on human pathological samples but also because of the development of different animal models of myelofibrosis. The aim of this review is to summarised the recent works performed on this disorder and more particularly to focus on the reactive stromal reaction mechanisms illustrated by the mice models.