FDA Approves Pivekimab Sunirine-pvzy (Decnupaz) for Blastic Plasmacytoid Dendritic Cell Neoplasm

On May 27, 2026, the U.S. Food and Drug Administration (FDA) approved pivekimab sunirine-pvzy (Decnupaz), a CD123-directed antibody and alkylating agent conjugate, for adults with blastic plasmacytoid dendritic cell neoplasm (BPDCN). The approval is supported by data from the multicenter, open-label, single-arm phase 1/2 CADENZA trial (NCT03386513), which included 33 treatment-naïve patients and 51 patients with relapsed or refractory disease.

Among the 33 treatment-naïve patients, 23 (69.7%; 95% CI, 51.3%-84.4%) achieved a complete remission or clinical complete remission (CR/CRc) with a median follow-up of 21.5 months; the median duration of CR/CRc was 9.7 months (95% CI, 2.9-not estimable). In the 51 relapsed or refractory patients, the CR/CRc rate was 15.7% (95% CI, 7.0%-28.6%) after a median follow-up of 24.1 months, with a median duration of CR/CRc of 9.2 months (range, 2.7-27.6+ months). Data published in the Journal of Clinical Oncology from the same trial showed an overall response rate of 80% in the 20-patient de novo primary analysis population and 85% in the broader 33-patient frontline cohort, with a median time to response of 1.4-1.5 months. In relapsed or refractory disease, the overall response rate was 35%.

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. In the trial, adverse events of grade 3 or higher occurred in 79% of patients; the most common any-grade adverse events were peripheral edema (54%), fatigue (26%), infusion-related reactions (26%), nausea (20%), and hypokalemia (20%). Two patients experienced vaso-occlusive disease; both recovered but discontinued treatment.

The recommended pivekimab sunirine-pvzy dose is 0.045 mg/kg administered intravenously over approximately 15-30 minutes once every three weeks (21-day cycle) until disease progression or unacceptable toxicity, calculated based on the patient's actual body weight.

The application was granted priority review, and pivekimab sunirine-pvzy received breakthrough therapy designation and orphan drug designation. The FDA review used the Assessment Aid, a voluntary submission from the applicant to facilitate assessment.

BPDCN is an ultra-rare and aggressive hematologic malignancy with limited treatment options. Historically, chemotherapy regimens adopted from acute leukemias have been used but are associated with poor outcomes and treatment-related toxicity; stem cell transplant is the only potentially curative therapy and often excludes older patients. Pivekimab sunirine is a first-in-class antibody-drug conjugate with high potency against tumor cells and is associated with less toxicity.

AbbVie, the drug's developer, has announced plans to present new data at the European Hematology Association (EHA) 2026 Congress, including a poster presentation on the efficacy of pivekimab sunirine in patients with BPDCN with baseline skin involvement from the CADENZA study.