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Myelofibrosis with myeloid metaplasia or idiopathic myelofibrosis is a myeloproliferative disease. It is known to be a stem-cell disorder that leads to a secondary and reactive stromal reaction in the bone marrow microenvironment that is responsible for impaired haematopoiesis. Although progress has been made in the elucidation of the pathogenesis of idiopathic myelofibrosis, lack of suitable models has limited our understanding of the pathology. The aim of this chapter is to address recent inferred new insights in mouse models into the pathogenesis of osteomyelofibrosis. These insights outline the role of transforming growth factor-beta1 and osteoprotegerin in the promotion of myelofibrosis and osteosclerosis, respectively, paying special regard to the role of abnormal megakaryocyte proliferation and maturation.

Original publication

DOI

10.1016/j.beha.2005.07.002

Type

Journal article

Journal

Best Pract Res Clin Haematol

Publication Date

2006

Volume

19

Pages

399 - 412

Keywords

Animals, Cytokines, Disease Models, Animal, Megakaryocytes, Mice, Osteosclerosis, Primary Myelofibrosis