On May 27, 2026, the FDA approved pivekimab sunirine-pvzy (Decnupaz) for adults with blastic plasmacytoid dendritic cell neoplasm (BPDCN), an ultra-rare hematologic malignancy. The approval was supported by the CADENZA study, a multicenter, open-label, single-arm trial that enrolled adults with treatment-
naive or relapsed/refractory BPDCN without evidence of active central nervous system disease. Efficacy was based on the rate of complete remission or clinical complete remission (CR/CRc).
Among patients with treatment-naive BPDCN, 69.7% achieved CR/CRc, with a median follow-up of 21.5 months. In patients with relapsed or refractory BPDCN, 15.7% achieved CR/CRc, with a median follow-up of 24.1 months. The median duration of response was 9.7 months in the treatment-naive group and 9.2 months in the relapsed/refractory group.
The recommended dose is 0.045 mg/kg intravenously over 15 to 30 minutes once every 3 weeks until disease progression or unacceptable toxicity. The prescribing information includes a boxed warning for hepatotoxicity, including hepatic veno-occlusive disease, and warnings and precautions for infusion-related reactions, edema, sulfite allergic reactions, and embryo-fetal toxicity.
Source
- US Food and Drug Administration. FDA approves pivekimab sunirine-pvzy for blastic plasmacytoid dendritic cell neoplasm, an ultra-rare hematologic malignancy. May 27, 2026. Accessed June 2, 2026. www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-pivekimab-sunirine-pvzy-blastic-plasmacytoid-dendritic-cell-neoplasm-ultra-rare