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Annals of Hematology
Published in: Annals of Hematology 11/2016

01-11-2016 | Review Article

Bone marrow niche in immune thrombocytopenia: a focus on megakaryopoiesis

Authors: Elaheh Khodadi, Ali Amin Asnafi, Saeid Shahrabi, Mohammad Shahjahani, Najmaldin Saki

Published in: Annals of Hematology | Issue 11/2016

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Abstract

Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by increased bleeding tendency and thrombocytopenia. In fact, the precise pathogenesis of this disease is still not clear. Megakaryopoiesis involves complete differentiation of megakaryocyte (MK) progenitors to functional platelets. This complex process occurs in specific bone marrow (BM) niches composed of several hematopoietic and non-hematopoietic cell types, soluble factors, and extracellular matrix proteins. These specialized microenvironments sustain MK maturation and localization to sinusoids as well as platelet release into circulation. However, MKs in ITP patients show impaired maturation and signs of degradation. Intrinsic defects in MKs and their extrinsic environment have been implicated in altered megakaryopoiesis in this disease. In particular, aberrant expression of miRNAs directing MK proliferation, differentiation, and platelet production; defective MK apoptosis; and reduced proliferation and differentiation rate of the MSC compartment observed in these patients may account for BM defects in ITP. Furthermore, insufficient production of thrombopoietin is another likely reason for ITP development. Therefore, identifying the signaling pathways and transcription factors influencing the interaction between MKs and BM niche in ITP patients will contribute to increased platelet production in order to prevent incomplete MK maturation and destruction as well as BM fibrosis and apoptosis in ITP. In this review, we will examine the interaction and role of BM niches in orchestrating megakaryopoiesis in ITP patients and discuss how these factors can be exploited to improve the quality of patient treatment and prognosis.
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Metadata
Title
Bone marrow niche in immune thrombocytopenia: a focus on megakaryopoiesis
Authors
Elaheh Khodadi
Ali Amin Asnafi
Saeid Shahrabi
Mohammad Shahjahani
Najmaldin Saki
Publication date
01-11-2016
Publisher
Springer Berlin Heidelberg
Published in
Annals of Hematology / Issue 11/2016
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-016-2703-1

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