FDA approves pivekimab sunirine-pvzy for blastic plasmacytoid dendritic cell neoplasm, an ultra-rare hematologic malignancy

Regulatory approval: On May 27, 2026, the FDA approved pivekimab sunirine-pvzy (Decnupaz; AbbVie), a CD123-directed antibody–alkylating agent conjugate, for adults with blastic plasmacytoid dendritic cell neoplasm (BPDCN). Approval was supported by the single-arm CADENZA trial (NCT03386513) evaluating CR/CRc in treatment-naïve and relapsed/refractory BPDCN without active CNS disease. The label includes a Boxed Warning for hepatotoxicity, including hepatic veno-occlusive disease.

FDA approval (May 27, 2026)

The US Food and Drug Administration (FDA) approved pivekimab sunirine-pvzy (Decnupaz; AbbVie, Inc.), a CD123-directed antibody and alkylating agent conjugate, for adults with blastic plasmacytoid dendritic cell neoplasm (BPDCN).

Full prescribing information will be posted on Drugs@FDA.

Supporting evidence: CADENZA (NCT03386513)

Efficacy and safety were evaluated in CADENZA, a multicenter, open-label, single-arm trial enrolling adults with treatment-naïve BPDCN (N=33) or relapsed/refractory BPDCN (N=51), without evidence of active CNS disease. The primary efficacy basis was complete remission or clinical complete remission (CR/CRc).

  • Treatment-naïve cohort (N=33): CR/CRc in 23 patients (69.7%; 95% CI, 51.3–84.4) at a median follow-up of 21.5 months. Median CR/CRc duration: 9.7 months (95% CI, 2.9–not estimable).
  • Relapsed/refractory cohort (N=51): CR/CRc in 8 patients (15.7%; 95% CI, 7.0–28.6) at a median follow-up of 24.1 months. Median CR/CRc duration: 9.2 months (range, 2.7–27.6+).

Key safety labeling

The prescribing information includes a Boxed Warning for hepatotoxicity, including hepatic veno-occlusive disease, and warnings/precautions for infusion-related reactions, edema, sulfite allergic reactions, and embryo-fetal toxicity.

Recommended dosage (label)

0.045 mg/kg IV over ~15–30 minutes once every 3 weeks (21-day cycle) until disease progression or unacceptable toxicity; dose is based on actual body weight.

The application received priority review and the product had breakthrough and orphan drug designations.

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