Critical review of pathogenetic mechanisms in myelofibrosis with myeloid metaplasia.
Myelofibrosis with myeloid metaplasia is an uncommon chronic myeloproliferative disorder characterized by extramedullary hematopoiesis associated with varying degrees of bone marrow fibrosis (hematopoiesis is clonal and fibrosis is a polyclonal reactive process). The primary defect in a pluripotent stem cell is still unknown. However, advances have been made during the past few years in the knowledge of the pathogenetic mechanisms in this disorder. This review focuses on current data on the cellular and molecular mechanisms possibly participating in the development of the disease.
Martyré
Marie-Claire
MC
INSERM U365, Institut Curie, 26 Rue d'Ulm, 75248 Paris, France. mmartyre@curie.fr
D006133:Growth Substances; D006410:Hematopoiesis; D006801:Humans; D039904:Pluripotent Stem Cells / Q000473:pathology; D055728:Primary Myelofibrosis / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D014157:Transcription Factors
D006133:Growth Substances; D014157:Transcription Factors