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Open Access 01-12-2023 | Primary Myelofibrosis | Review

Fibrosis and bone marrow: understanding causation and pathobiology

Authors: Kanjaksha Ghosh, Durjoy K. Shome, Bipin Kulkarni, Malay K. Ghosh, Kinjalka Ghosh

Published in: Journal of Translational Medicine | Issue 1/2023

Abstract

Bone marrow fibrosis represents an important structural change in the marrow that interferes with some of its normal functions. The aetiopathogenesis of fibrosis is not well established except in its primary form. The present review consolidates current understanding of marrow fibrosis. We searched PubMed without time restriction using key words: bone marrow and fibrosis as the main stem against the terms: growth factors, cytokines and chemokines, morphology, megakaryocytes and platelets, myeloproliferative disorders, myelodysplastic syndrome, collagen biosynthesis, mesenchymal stem cells, vitamins and minerals and hormones, and mechanism of tissue fibrosis. Tissue marrow fibrosis-related papers were short listed and analysed for the review. It emerged that bone marrow fibrosis is the outcome of complex interactions between growth factors, cytokines, chemokines and hormones together with their facilitators and inhibitors. Fibrogenesis is initiated by mobilisation of special immunophenotypic subsets of mesenchymal stem cells in the marrow that transform into fibroblasts. Fibrogenic stimuli may arise from neoplastic haemopoietic or non-hematopoietic cells, as well as immune cells involved in infections and inflammatory conditions. Autoimmunity is involved in a small subset of patients with marrow fibrosis. Megakaryocytes and platelets are either directly involved or are important intermediaries in stimulating mesenchymal stem cells. MMPs, TIMPs, TGF-β, PDGRF, and basic FGF and CRCXL4 chemokines are involved in these processes. Genetic and epigenetic changes underlie many of these conditions.

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Title: Fibrosis and bone marrow: understanding causation and pathobiology
Authors: Kanjaksha Ghosh
Durjoy K. Shome
Bipin Kulkarni
Malay K. Ghosh
Kinjalka Ghosh
Publication date: 01-12-2023
Publisher: BioMed Central
Keywords: Primary Myelofibrosis
Primary Myelofibrosis
Targeted Therapy
Cytokines
Myelodysplastic Syndrome
Published in: Journal of Translational Medicine / Issue 1/2023
Electronic ISSN: 1479-5876
DOI: https://doi.org/10.1186/s12967-023-04393-z

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