TY - JOUR
T1 - Fibrogenesis in primary myelofibrosis
T2 - Diagnostic, clinical, and therapeutic implications
AU - Nazha, Aziz
AU - Khoury, Joseph D.
AU - Rampal, Raajit K.
AU - Daver, Naval
N1 - Publisher Copyright:
© AlphaMed Press 2015.
PY - 2015/8/24
Y1 - 2015/8/24
N2 - 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.
AB - 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.
KW - Bone marrow fibrosis
KW - Collagen fibrosis
KW - Primary myelofibrosis
KW - Reticulin fibrosis
UR - http://www.scopus.com/inward/record.url?scp=84943271301&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84943271301&partnerID=8YFLogxK
U2 - 10.1634/theoncologist.2015-0094
DO - 10.1634/theoncologist.2015-0094
M3 - Article
C2 - 26304912
AN - SCOPUS:84943271301
SN - 1083-7159
VL - 20
SP - 1154
EP - 1160
JO - Oncologist
JF - Oncologist
IS - 10
ER -