Fibrogenesis in primary myelofibrosis: Diagnostic, clinical, and therapeutic implications Review uri icon

Overview

MeSH Major

  • Bone Marrow Cells
  • Primary Myelofibrosis

abstract

  • Primary myelofibrosis is a stem cell-derived clonal malignancy characterized by unchecked proliferation of myeloid cells, resulting in bone marrow fibrosis, osteosclerosis, and pathologic angiogenesis. Bone marrow fibrosis (BMF) plays a central role in the pathophysiology of the disease. This review describes current issues regarding BMF in primary myelofibrosis, including the pathophysiology and impact of abnormal deposition of excess collagen and reticulin fibers in bone marrow spaces, the modified Bauermeister and the European Consensus grading systems of BMF, and the prognostic impact of BMF on the overall outcome of patients with primary myelofibrosis. The impact of novel therapeutic strategies, including JAK-STAT inhibitors and allogeneic stem cell transplant, on BMF is discussed.

publication date

  • August 24, 2015

Research

keywords

  • Review

Identity

Language

  • eng

PubMed Central ID

  • PMC4591957

Digital Object Identifier (DOI)

  • 10.1634/theoncologist.2015-0094

PubMed ID

  • 26304912

Additional Document Info

start page

  • 1154

end page

  • 60

volume

  • 20

number

  • 10