title: [Pathomechanism and clinical impact of myelofibrosis in neoplastic diseases of the bone marrow].
abstract: Polyclonal mesenchymal cells (fibroblasts, endothelial cells, pericytes, osteoblasts, reticular cells, adipocytes, etc.) of the bone marrow create a functional microenvironment, which actively contributes to the maintenance of hemopoesis. This takes place through cellular interactions via growth factors, cytokines, adhesion molecules and extracellular matrix components, as well as through the control of calcium and oxygen concentration. Inflammatory and neoplastic diseases of the bone marrow result in pathologic interaction between hemopoietic progenitors and stromal cells. This may lead to the activation and expansion of the stroma and to the accumulation of reticulin and collagen fibers produced by mesenchymal cells. Clinically relevant fiber accumulation, termed as myelofibrosis accompanies many diseases, although, the extent and the consequence of myelofibrosis are variable in different disorders. The aim of this review is to summarize basic features of the normal bone marrow mesenchymal environment and the pathological process leading to myelofibrosis. In addition, the special features of myelofibrosis in bone marrow diseases, including myeloproliferative neoplasia, myelodysplastic syndrome and other neoplastic conditions are discussed.
authors.affiliation: Debreceni Egyetem, Általános Orvostudományi Kar Pathologiai Intézet Debrecen Nagyerdei krt. 98. 4012.
authors.forename: Judit
authors.initials: J
authors.lastname: Bedekovics
authors.affiliation: Debreceni Egyetem, Általános Orvostudományi Kar Pathologiai Intézet Debrecen Nagyerdei krt. 98. 4012.
authors.forename: Gábor
authors.initials: G
authors.lastname: Méhes
mesh_terms: D000208:Acute Disease; D001853:Bone Marrow / Q000378:metabolism*; D019046:Bone Marrow Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology*; D002118:Calcium / Q000378:metabolism; D015815:Cell Adhesion Molecules / Q000378:metabolism; D003094:Collagen / Q000378:metabolism; D016207:Cytokines / Q000378:metabolism; D005109:Extracellular Matrix / Q000378:metabolism; D006410:Hematopoiesis; D006412:Hematopoietic Stem Cells / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007938:Leukemia / Q000473:pathology / Q000503:physiopathology; D059630:Mesenchymal Stem Cells / Q000378:metabolism / Q000473:pathology; D009190:Myelodysplastic Syndromes / Q000473:pathology / Q000503:physiopathology; D010100:Oxygen / Q000378:metabolism; D055728:Primary Myelofibrosis / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology*; D012155:Reticulin / Q000378:metabolism
chemical_list: D015815:Cell Adhesion Molecules; D016207:Cytokines; D012155:Reticulin; D003094:Collagen; D010100:Oxygen; D002118:Calcium
keywords: bone marrow; csontvelő; microenvironment; mikrokörnyezet; myelofibrosis