CD123, also known as the interleukin-3 receptor alpha chain (IL-3Rα), is a glycoprotein composed of 360 amino acids. It forms a high-affinity IL-3 receptor with CD131, and by binding to the IL-3 ligand, it promotes the proliferation and survival of hematopoietic stem/progenitor cells (HSPCs). In various hematological malignancies, such as acute myeloid leukemia (AML), blastic plasmacytoid dendritic cell neoplasm (BPDCN), acute lymphoblastic leukemia/lymphoma (ALL), hairy cell leukemia (HCL), and systemic mastocytosis (SM), the expression level of CD123 is often high and associated with the overproliferation of tumor cells. It is worth noting that the expression level of CD123 increases with the progression and worsening of hematological malignancies, indicating that the expression level of CD123 may be related to the severity of the disease. Moreover, high expression of CD123 is associated with poor prognosis in AML, making it an ideal target for AML immunotherapy.