Fibrogenesis in primary myelofibrosis: Diagnostic, clinical, and therapeutic implications Journal Article
| Authors: | Nazha, A.; Khoury, J. D.; Rampal, R. K.; Daver, N. |
| Article Title: | Fibrogenesis in primary myelofibrosis: Diagnostic, clinical, and therapeutic implications |
| 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. © AlphaMed Press 2015. |
| Keywords: | platelet derived growth factor; vasculotropin; protein expression; myelofibrosis; gene mutation; busulfan; allogeneic stem cell transplantation; myeloid metaplasia; primary myelofibrosis; nonhuman; pathophysiology; outcome assessment; transforming growth factor beta; differential diagnosis; gelatinase b; interleukin 8; hemoglobin; protein tyrosine kinase inhibitor; fibroblast growth factor 2; tumor necrosis factor alpha; bone marrow biopsy; acute myeloblastic leukemia; collagen; thrombocyte count; leukocyte count; lymphocytic infiltration; megakaryocyte; recombinant granulocyte macrophage colony stimulating factor; myeloproliferative neoplasm; thrombopoietin; randomized controlled trial (topic); recombinant alpha2b interferon; autoimmune lymphoproliferative syndrome; reticulin; ruxolitinib; human; priority journal; article; bone marrow fibrosis; fibrogenesis; collagen fibrosis; reticulin fibrosis |
| Journal Title: | The Oncologist |
| Volume: | 20 |
| Issue: | 10 |
| ISSN: | 1083-7159 |
| Publisher: | Oxford University Press |
| Date Published: | 2015-10-01 |
| Start Page: | 1154 |
| End Page: | 1160 |
| Language: | English |
| DOI: | 10.1634/theoncologist.2015-0094 |
| PROVIDER: | scopus |
| PMCID: | PMC4591957 |
| PUBMED: | 26304912 |
| DOI/URL: | |
| Notes: | Export Date: 2 November 2015 -- Source: Scopus |
