On May 27, 2026, the U.S. Food and Drug Administration (FDA) approved pivekimab sunirine-pvzy (Decnupaz), a CD123-directed antibody-drug conjugate delivering a DNA alkylating payload, for the treatment of adults with blastic plasmacytoid dendritic cell neoplasm (BPDCN).¹
The approval was based on the phase 1/2 CADENZA study (NCT03386513), a multicenter, open-label, single-arm trial of 84 adults with BPDCN without evidence of active central nervous system disease. The study included 33 patients with treatment-naïve BPDCN and 51 patients with relapsed or refractory disease. The primary efficacy endpoint was complete remission or clinical complete remission (CR/CRc). Among patients with treatment-naïve BPDCN, 23 of 33 patients achieved CR/CRc (69.7%; 95% CI, 51.3%-84.4%), with a median duration of CR/CRc of 9.7 months. Among patients with relapsed or refractory BPDCN, 8 of 51 patients achieved CR/CRc (15.7%; 95% CI, 7.0%-28.6%), with a median duration of CR/CRc of 9.2 months.¹,²
In CADENZA, the most common adverse events included peripheral edema (54%), fatigue (26%), and infusion-related reactions (26%). Grade 3 or higher adverse events included neutropenia (16%), thrombocytopenia (14%), and peripheral edema (12%).² 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.¹
References
- US Food and Drug Administration. FDA approves pivekimab sunirine-pvzy for blastic plasmacytoid dendritic cell neoplasm, an ultra-rare hematologic malignancy. Published May 27, 2026. Accessed May 28, 2026. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-pivekimab-sunirine-pvzy-blastic-plasmacytoid-dendritic-cell-neoplasm-ultra-rare
- Pemmaraju N, Marconi G, Montesinos P, et al. Pivekimab sunirine in blastic plasmacytoid dendritic cell neoplasm. J Clin Oncol. 2026;44(10):861-873. doi:10.1200/JCO-25-02083.