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Megakaryocyte Contribution to Bone Marrow Fibrosis: many Arrows in the Quiver

In Primary Myelofibrosis (PMF), megakaryocyte dysplasia/hyperplasia determines the release of inflammatory cytokines that, in turn, stimulate stromal cells and induce bone marrow fibrosis. The pathogenic mechanism and the cells responsible for progression to bone marrow fibrosis in PMF are not compl...

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Detalles Bibliográficos
Autores principales: Malara, Alessandro, Abbonante, Vittorio, Zingariello, Maria, Migliaccio, Annarita, Balduini, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6223581/
https://www.ncbi.nlm.nih.gov/pubmed/30416700
http://dx.doi.org/10.4084/MJHID.2018.068
Descripción
Sumario:In Primary Myelofibrosis (PMF), megakaryocyte dysplasia/hyperplasia determines the release of inflammatory cytokines that, in turn, stimulate stromal cells and induce bone marrow fibrosis. The pathogenic mechanism and the cells responsible for progression to bone marrow fibrosis in PMF are not completely understood. This review article aims to provide an overview of the crucial role of megakaryocytes in myelofibrosis by discussing the role and the altered secretion of megakaryocyte-derived soluble factors, enzymes and extracellular matrices that are known to induce bone marrow fibrosis.