Inflammatory Pathways and the Bone Marrow Microenvironment in Inherited Bone Marrow Failure Syndromes.

IF 4 2区 医学 Q2 BIOTECHNOLOGY & APPLIED MICROBIOLOGY
STEM CELLS Pub Date : 2025-04-29 DOI:10.1093/stmcls/sxaf021
Nicholas Neoman, Hye Na Kim, Jacob Viduya, Anju Goyal, Y Lucy Liu, Kathleen M Sakamoto
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引用次数: 0

Abstract

Inherited Bone Marrow Failure Syndromes (IBMFS) are a diverse group of genetic disorders characterized by insufficient hematopoietic cell production due to blood stem cell dysfunction. The most common syndromes are Fanconi Anemia, Diamond-Blackfan Anemia, and Shwachman-Diamond Syndrome. These conditions share a theme of chronically producing pro-inflammatory cytokines such as TNF-α, IL-1β, IL-6, TGF-β, IFN-I, and IFN-γ. Each of these cytokines can impact the bone marrow microenvironment and drive the pathophysiology of IBMFS. This review aims to provide the latest progress in the field regarding the mechanistic underpinnings of inflammation in these IBMFS, as well as the effect of inflammation on the bone marrow microenvironment. A comprehensive understanding of the inflammation in IBMFS will open new avenues for intervention to restore bone marrow stability and improve patient prognosis. Future research must include targeting these mechanisms to develop novel therapies that can potentially mitigate the effects of chronic inflammation in IBMFS.

炎症途径和骨髓微环境在遗传性骨髓衰竭综合征。
遗传性骨髓衰竭综合征(IBMFS)是一组多样化的遗传性疾病,其特征是由于造血干细胞功能障碍导致造血细胞产生不足。最常见的综合征是范可尼贫血症、Diamond-Blackfan贫血症和Shwachman-Diamond综合征。这些疾病都有一个共同的主题,即慢性产生促炎细胞因子,如TNF-α、IL-1β、IL-6、TGF-β、IFN- i和IFN-γ。这些细胞因子都可以影响骨髓微环境并驱动IBMFS的病理生理。本文旨在就炎症在IBMFS中的机制基础以及炎症对骨髓微环境的影响等方面的最新进展进行综述。对IBMFS炎症的全面了解将为恢复骨髓稳定性和改善患者预后开辟新的干预途径。未来的研究必须包括针对这些机制来开发可能减轻IBMFS慢性炎症影响的新疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
STEM CELLS
STEM CELLS 医学-生物工程与应用微生物
CiteScore
10.30
自引率
1.90%
发文量
104
审稿时长
3 months
期刊介绍: STEM CELLS, a peer reviewed journal published monthly, provides a forum for prompt publication of original investigative papers and concise reviews. STEM CELLS is read and written by clinical and basic scientists whose expertise encompasses the rapidly expanding fields of stem and progenitor cell biology. STEM CELLS covers: Cancer Stem Cells, Embryonic Stem Cells/Induced Pluripotent Stem (iPS) Cells, Regenerative Medicine, Stem Cell Technology: Epigenetics, Genomics, Proteomics, and Metabonomics, Tissue-Specific Stem Cells, Translational and Clinical Research.
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