The role of RNA editing in hematopoietic system. An increasing body of research indicates various roles of RNA editing in the hematopoietic system. The most classical role of RNA editing involves regulating the immune response, specifically by adding inosine to dsRNAs to prevent the activation of the MDA5/MAVS signaling pathway, thereby avoiding the activation of IFN and ISGs. RNA editing is also crucial for maintaining hematopoietic homeostasis, particularly because a normal RNA editome ensures the proper function of HSPCs and guarantees normal lineage commitment. Additionally, in response to external stimuli such as hypoxia, temperature changes, or inflammation, RNA editing (primarily C-to-U) helps cells adapt. During cell aging, upregulated ADAR1 p150 can accelerate the aging of HSPCs, promote myeloid lineage skewing, and modulate inflammation-responsive transcription factors (TFs). Finally, in the case of abnormal hematopoiesis, under the influence of the inflammatory environment within the bone marrow niche, we find that tumor cells upregulate ADAR expression, leading to aberrant RNA editing events (such as Azin1, STAT3, MDM2, pri-let7, and pri-miR-26a). These altered RNA editing patterns cause dysregulation of the cell cycle and contribute to the proliferation of LSCs, promoting treatment resistance and poor prognosis. The human population and mouse icons represent the sources of the studied samples. TNF-α, tumor necrosis factor-α.