To the editor;
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy derived from a specific subset of dendritic cells involving skin, bone marrow and other lymphoid organs. To date both acute lymphoblastic and myeloblastic leukemia treatment regimens and FLAG-IDA and hyper CYVAD regimens have been tried but nonetheless disease free survival is low. Tagraxofusp-erz is an FDA approved recombinant cytotoxin as the first CD123-targeted agent ever approved for BPDCN (1). Another agent; Venetoclax, a bcl-2 inhibitor, is being used for relapsed – refractory AML and there are encouraging results as BPDCN survival depends on anti-apopitotic activity protein bcl-2 which was shown in recent studies (2). Here we presented a pediatric case with recurrent BPDCN, first pediatric case of combination therapy with tagraxofusp plus venetoclax and azacitidine plus venetoclax for BPDCN.