Combination of tagraxofusp and azacitidine is an effective option for relapsed blastic plasmacytoid dendritic cell neoplasm after allogeneic hematopoietic stem cell transplantation

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and clinically aggressive hematologic malignancy with historically dismal outcomes despite intensive chemotherapy including allogeneic hematopoietic stem cell transplantation (allo-HSCT). We here report on a successful treatment approach with tagraxofusp and azacitidine in an older patient with relapsed BPDCN four months after allo-HSCT. The combined therapy was well tolerated without any serious adverse events including capillary leak syndrome or exacerbation of graft-versus-host disease. Positron emission tomography–computed tomography showed a complete metabolic remission after two treatment cycles. This combination treatment may therefore become the treatment of choice for patients with BPDCN and relapse after allo-HSCT.