Constitutive activation of the NF-κB signaling pathway within BPDCN leads to overexpression of BCL-2.[49] Venetoclax is a selective BCL-2 inhibitor that has demonstrated clinical activity in a variety of hematologic malignancies, including chronic lymphocytic leukemia, myelodysplastic syndromes, and AML.[45] Recently, Montero et al[65] demonstrated in a BPDCN cell line with a BPDCN patient-derived xenograft mouse model that BPDCN is highly dependent on the anti-apoptotic protein BCL-2 and significantly sensitive to venetoclax, which effectively increases mouse survival. Here, NFKB1 is linked to CD4+/CD56+ hematodermic neoplasm.