Myelodysplasia is in the niche: novel concepts and emerging therapies.
Myelodysplasia is in the niche: novel concepts and emerging therapies.
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作者：
骨髓增生异常综合征（MDS）是一种主要发生于老年人的克隆性疾病，其特征是造血功能低下，转化为急性髓性白血病的风险增加。MDS的发病机制被认为是从特定的遗传或表观遗传事件的积累和选择演变而来的。新出现的证据表明，MDS不仅仅是一种造血系统疾病，而是影响整个骨髓微环境，包括骨代谢。这些细胞中的许多，特别是间充质干细胞和祖细胞（MSPC）和成骨细胞，表达许多粘附分子和分泌因子，其通过促进造血干细胞和祖细胞（HSPC）维持、自我更新和分化来调节整个生命中的血液再生。一些内分泌因子，如促红细胞生成素、甲状旁腺激素和雌激素，以及紊乱的铁代谢调节这些过程。因此，MSPC和HSPC之间的相互作用有助于MDS的发病机制和相关病理。详细了解这些机制可能有助于确定新的诊断和治疗目标。在这篇综述中，我们将讨论“骨血液学”作为MDS的一个新兴研究领域的科学原理，并概述其临床意义。
Myelodysplastic syndromes (MDSs) represent clonal disorders mainly of the elderly that are characterized by ineffective hematopoiesis and an increased risk of transformation into acute myeloid leukemia. The pathogenesis of MDS is thought to evolve from accumulation and selection of specific genetic or epigenetic events. Emerging evidence indicates that MDS is not solely a hematopoietic disease but rather affects the entire bone marrow microenvironment, including bone metabolism. Many of these cells, in particular mesenchymal stem and progenitor cells (MSPCs) and osteoblasts, express a number of adhesion molecules and secreted factors that regulate blood regeneration throughout life by contributing to hematopoietic stem and progenitor cell (HSPC) maintenance, self-renewal and differentiation. Several endocrine factors, such as erythropoietin, parathyroid hormone and estrogens, as well as deranged iron metabolism modulate these processes. Thus, interactions between MSPC and HSPC contribute to the pathogenesis of MDS and associated pathologies. A detailed understanding of these mechanisms may help to define novel targets for diagnosis and possibly therapy. In this review, we will discuss the scientific rationale of ‘osteohematology' as an emerging research field in MDS and outline clinical implications.
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影响因子：
82.9
作者：
Dussiot M;Maciel TT;Fricot A;Chartier C;Negre O;Veiga J;Grapton D;Paubelle E;Payen E;Beuzard Y;Leboulch P;Ribeil JA;Arlet JB;Coté F;Courtois G;Ginzburg YZ;Daniel TO;Chopra R;Sung V;Hermine O;Moura IC
通讯作者：
Moura IC
影响因子：
4.8
作者：
Cho, Sun Wook;Pirih, Flavia Q.;McCauley, Laurie K.
通讯作者：
McCauley, Laurie K.
影响因子：
--
作者：
Churchman, Sarah M.;Ponchel, Frederique;Jones, Elena A.
通讯作者：
Jones, Elena A.
影响因子：
15.9
作者：
Babitt, Jodie L.;Huang, Franklin W.;Lin, Herbert Y.
通讯作者：
Lin, Herbert Y.
DOI：
10.1084/jem.20131141
发表时间：
2013-11-18
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Abdel-Wahab O;Gao J;Adli M;Dey A;Trimarchi T;Chung YR;Kuscu C;Hricik T;Ndiaye-Lobry D;Lafave LM;Koche R;Shih AH;Guryanova OA;Kim E;Li S;Pandey S;Shin JY;Telis L;Liu J;Bhatt PK;Monette S;Zhao X;Mason CE;Park CY;Bernstein BE;Aifantis I;Levine RL
通讯作者：
Levine RL