Focus on Osteosclerotic Progression in Primary Myelofibrosis
Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hematopoietic stem-cell-derived clonal proliferation, leading to bone marrow (BM) fibrosis. Hematopoiesis alterations are closely associated with modifications of the BM microenvironment, characterized by defective interac...
| Main Authors: | , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-01-01
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| Series: | Biomolecules |
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| Online Access: | https://www.mdpi.com/2218-273X/11/1/122 |
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| author | Mariarita Spampinato Cesarina Giallongo Alessandra Romano Lucia Longhitano Enrico La Spina Roberto Avola Grazia Scandura Ilaria Dulcamare Vincenzo Bramanti Michelino Di Rosa Nunzio Vicario Rosalba Parenti Giovanni Li Volti Daniele Tibullo Giuseppe A. Palumbo |
| author_facet | Mariarita Spampinato Cesarina Giallongo Alessandra Romano Lucia Longhitano Enrico La Spina Roberto Avola Grazia Scandura Ilaria Dulcamare Vincenzo Bramanti Michelino Di Rosa Nunzio Vicario Rosalba Parenti Giovanni Li Volti Daniele Tibullo Giuseppe A. Palumbo |
| author_sort | Mariarita Spampinato |
| collection | DOAJ |
| description | Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hematopoietic stem-cell-derived clonal proliferation, leading to bone marrow (BM) fibrosis. Hematopoiesis alterations are closely associated with modifications of the BM microenvironment, characterized by defective interactions between vascular and endosteal niches. As such, neoangiogenesis, megakaryocytes hyperplasia and extensive bone marrow fibrosis, followed by osteosclerosis and bone damage, are the most relevant consequences of PMF. Moreover, bone tissue deposition, together with progressive fibrosis, represents crucial mechanisms of disabilities in patients. Although the underlying mechanisms of bone damage observed in PMF are still unclear, the involvement of cytokines, growth factors and bone marrow microenvironment resident cells have been linked to disease progression. Herein, we focused on the role of megakaryocytes and their alterations, associated with cytokines and chemokines release, in modulating functions of most of the bone marrow cell populations and in creating a complex network where impaired signaling strongly contributes to progression and disabilities. |
| first_indexed | 2024-03-09T04:23:27Z |
| format | Article |
| id | doaj.art-f5bfe06bcda94a9795bf844533391336 |
| institution | Directory Open Access Journal |
| issn | 2218-273X |
| language | English |
| last_indexed | 2024-03-09T04:23:27Z |
| publishDate | 2021-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomolecules |
| spelling | doaj.art-f5bfe06bcda94a9795bf8445333913362023-12-03T13:44:42ZengMDPI AGBiomolecules2218-273X2021-01-0111112210.3390/biom11010122Focus on Osteosclerotic Progression in Primary MyelofibrosisMariarita Spampinato0Cesarina Giallongo1Alessandra Romano2Lucia Longhitano3Enrico La Spina4Roberto Avola5Grazia Scandura6Ilaria Dulcamare7Vincenzo Bramanti8Michelino Di Rosa9Nunzio Vicario10Rosalba Parenti11Giovanni Li Volti12Daniele Tibullo13Giuseppe A. Palumbo14Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalyDepartment of Medical and Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, ItalyDepartment of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, ItalySection of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalyDepartment of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, ItalySection of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalyDepartment of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, ItalyDepartment of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, ItalyDivision of Clinical Pathology, “Giovanni Paolo II” Hospital–A.S.P. Ragusa, 97100 Ragusa, ItalySection of Human Anatomy and Histology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalySection of Physiology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalySection of Physiology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalySection of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalySection of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, ItalyDepartment of Medical and Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, ItalyPrimary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by hematopoietic stem-cell-derived clonal proliferation, leading to bone marrow (BM) fibrosis. Hematopoiesis alterations are closely associated with modifications of the BM microenvironment, characterized by defective interactions between vascular and endosteal niches. As such, neoangiogenesis, megakaryocytes hyperplasia and extensive bone marrow fibrosis, followed by osteosclerosis and bone damage, are the most relevant consequences of PMF. Moreover, bone tissue deposition, together with progressive fibrosis, represents crucial mechanisms of disabilities in patients. Although the underlying mechanisms of bone damage observed in PMF are still unclear, the involvement of cytokines, growth factors and bone marrow microenvironment resident cells have been linked to disease progression. Herein, we focused on the role of megakaryocytes and their alterations, associated with cytokines and chemokines release, in modulating functions of most of the bone marrow cell populations and in creating a complex network where impaired signaling strongly contributes to progression and disabilities.https://www.mdpi.com/2218-273X/11/1/122primary myelofibrosisbonemyeloproliferative neoplasmbone marrowfibrosis |
| spellingShingle | Mariarita Spampinato Cesarina Giallongo Alessandra Romano Lucia Longhitano Enrico La Spina Roberto Avola Grazia Scandura Ilaria Dulcamare Vincenzo Bramanti Michelino Di Rosa Nunzio Vicario Rosalba Parenti Giovanni Li Volti Daniele Tibullo Giuseppe A. Palumbo Focus on Osteosclerotic Progression in Primary Myelofibrosis Biomolecules primary myelofibrosis bone myeloproliferative neoplasm bone marrow fibrosis |
| title | Focus on Osteosclerotic Progression in Primary Myelofibrosis |
| title_full | Focus on Osteosclerotic Progression in Primary Myelofibrosis |
| title_fullStr | Focus on Osteosclerotic Progression in Primary Myelofibrosis |
| title_full_unstemmed | Focus on Osteosclerotic Progression in Primary Myelofibrosis |
| title_short | Focus on Osteosclerotic Progression in Primary Myelofibrosis |
| title_sort | focus on osteosclerotic progression in primary myelofibrosis |
| topic | primary myelofibrosis bone myeloproliferative neoplasm bone marrow fibrosis |
| url | https://www.mdpi.com/2218-273X/11/1/122 |
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