Abstract
Immune-mediated bone marrow failure syndromes (BMFS) are characterized by ineffective marrow haemopoiesis and subsequent peripheral cytopenias. Ineffective haemopoiesis is the result of a complex marrow deregulation including genetic, epigenetic, and immune-mediated alterations in haemopoietic stem/progenitor cells, as well as abnormal haemopoietic-to-stromal cell interactions, with abnormal release of haemopoietic growth factors, chemokines, and inhibitors. Mesenchymal stem/stromal cells (MSCs) and their progeny (i.e., osteoblasts, adipocytes, and reticular cells) are considered as key cellular components of the bone marrow haemopoietic niche. MSCs may interfere with haemopoietic as well as immune regulation. Evidence suggests that bone marrow MSCs may be involved in immune-mediated BMFS underlying pathophysiology, harboring either native abnormalities and/or secondary defects, caused by exposure to activated marrow components. This review summarizes previous as well as more recent information related to the biologic/functional characteristics of bone marrow MSCs in myelodysplastic syndromes, acquired aplastic anemia, and chronic idiopathic neutropenia.
Highlights
Immune-mediated bone marrow failure syndromes (BMFS), such as the myelodysplastic syndromes, acquired aplastic anemia, or chronic idiopathic neutropenia, are characterized by ineffective marrow haemopoiesis and subsequent peripheral cytopenias
Osteoblasts, adipocytes, and reticular cells of the marrow stroma derive from a common progenitor cell, the mesenchymal stem/stromal cell (MSC) [1,2,3,4,5]
The purpose of this review is to summarize and discuss literature information regarding the biologic and functional characteristics of BM MSCs in the immune-mediated BMFS, namely, myelodysplastic syndromes, chronic idiopathic neutropenia, and aplastic anemia
Summary
Immune-mediated bone marrow failure syndromes (BMFS), such as the myelodysplastic syndromes, acquired aplastic anemia, or chronic idiopathic neutropenia, are characterized by ineffective marrow haemopoiesis and subsequent peripheral cytopenias. Pathogenetic mechanisms involve a complex marrow deregulation, including genetic and epigenetic alterations, resulting in aberrant release of haemopoietic growth factors and inhibitors in the marrow, deregulated immune manifestations, all resulting in defective haemopoietic maturation and increased haemopoietic cell apoptosis. Key cellular components of the bone marrow (BM) haemopoietic microenvironment include osteoblasts, sinusoidal endothelial cells, macrophages, adipocytes, and reticular cells, orchestrating the maintenance, proliferation, and differentiation of haemopoietic stem and progenitor cells (HSPCs). The purpose of this review is to summarize and discuss literature information regarding the biologic and functional characteristics of BM MSCs in the immune-mediated BMFS, namely, myelodysplastic syndromes, chronic idiopathic neutropenia, and aplastic anemia
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
