骨髓纤维化代表骨髓中的重要结构变化,其干扰其一些正常功能。除主要形式外,纤维化的病因尚未完全确定。本综述巩固了目前对骨髓纤维化的理解。我们在没有时间限制的情况下搜索PubMed,使用关键词:骨髓和纤维化作为主要词干,而不是生长因子,细胞因子和趋化因子,形态学,巨核细胞和血小板,骨髓增生性疾病,骨髓增生异常综合征,胶原蛋白生物合成,间充质干细胞,维生素、矿物质和荷尔蒙,和组织纤维化的机制。组织骨髓纤维化相关论文入围并进行分析以进行综述。发现骨髓纤维化是生长因子之间复杂相互作用的结果,细胞因子,趋化因子和激素以及它们的促进剂和抑制剂。纤维化是通过动员骨髓中转化为成纤维细胞的间充质干细胞的特殊免疫表型亚群而启动的。纤维化刺激可能来自肿瘤造血细胞或非造血细胞,以及参与感染和炎症的免疫细胞。自身免疫涉及一小部分骨髓纤维化患者。巨核细胞和血小板直接参与刺激间充质干细胞或作为重要的中介。MMPs,TIMP,TGF-β,PDGRF,碱性FGF和CRCXL4趋化因子参与这些过程。遗传和表观遗传变化是许多这些疾病的基础。
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.