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.
authors: Leukemia Program, Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland, Ohio USA; Department of Hematopathology, and Leukemia Department, The University of Texas MD Anderson Cancer Center, Houston, Texas USA; Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA nazhaa@ccf.org.
authors: Aziz
authors: A
authors: Nazha
authors: Leukemia Program, Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland, Ohio USA; Department of Hematopathology, and Leukemia Department, The University of Texas MD Anderson Cancer Center, Houston, Texas USA; Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
authors: Joseph D
authors: JD
authors: Khoury
authors: Leukemia Program, Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland, Ohio USA; Department of Hematopathology, and Leukemia Department, The University of Texas MD Anderson Cancer Center, Houston, Texas USA; Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
authors: Raajit K
authors: RK
authors: Rampal
authors: Leukemia Program, Department of Hematology and Medical Oncology, Taussig Cancer Institute, Cleveland, Ohio USA; Department of Hematopathology, and Leukemia Department, The University of Texas MD Anderson Cancer Center, Houston, Texas USA; Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA.
authors: Naval
authors: N
authors: Daver
mesh_terms: D001854:Bone Marrow Cells / Q000473:pathology*; D002066:Busulfan / Q000627:therapeutic use; D005355:Fibrosis / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D053612:Janus Kinases / Q000037:antagonists & inhibitors; D009570:Nitriles; D055728:Primary Myelofibrosis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D011379:Prognosis; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines; D050791:STAT Transcription Factors / Q000037:antagonists & inhibitors; D033581:Stem Cell Transplantation / Q000379:methods
chemical_list: D009570:Nitriles; D011720:Pyrazoles; D011743:Pyrimidines; D050791:STAT Transcription Factors; C540383:ruxolitinib; D053612:Janus Kinases; D002066:Busulfan
keywords: Bone marrow fibrosis; Collagen fibrosis; Primary myelofibrosis; Reticulin fibrosis