Focus on Osteosclerotic Progression in Primary Myelofibrosis
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
Affiliations
Mariarita Spampinato
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Cesarina Giallongo
Department of Medical and Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, Italy
Alessandra Romano
Department of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, Italy
Lucia Longhitano
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Enrico La Spina
Department of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, Italy
Roberto Avola
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Grazia Scandura
Department of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, Italy
Ilaria Dulcamare
Department of General Surgery and Medical-Surgical Specialties, Division of Hematology, A.O.U. “Policlinico-Vittorio Emanuele”, University of Catania, 95123 Catania, Italy
Vincenzo Bramanti
Division of Clinical Pathology, “Giovanni Paolo II” Hospital–A.S.P. Ragusa, 97100 Ragusa, Italy
Michelino Di Rosa
Section of Human Anatomy and Histology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Nunzio Vicario
Section of Physiology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Rosalba Parenti
Section of Physiology, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Giovanni Li Volti
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Daniele Tibullo
Section of Biochemistry, Department of Biomedical and Biotechnological Sciences, University of Catania, 95123 Catania, Italy
Giuseppe A. Palumbo
Department of Medical and Surgical Sciences and Advanced Technologies “G.F. Ingrassia”, University of Catania, 95123 Catania, Italy
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.
