Loading
10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Blastic plasmacytoid dendritic cell neoplasm is a rare aggressive leukaemia-like blood cancer of dendritic-cell precursors, a few hundred US cases a year, that often first appears as bruise-like skin lesions. Two CD123-directed drugs, tagraxofusp and pivekimab sunirine, are the first targeted therapies; allogeneic transplant in first remission is still the only route to long-term control.
BPDCN derives from plasmacytoid dendritic cell precursors, expresses CD4, CD56, CD123 (IL-3 receptor alpha), TCF4 and TCL1, and presents with skin lesions, marrow involvement and cytopenias, often evolving to a leukaemic phase. Historically it was treated with ALL- or AML-type chemotherapy with brief responses and a median survival around a year.
Tagraxofusp (CD123-directed IL-3/diphtheria toxin fusion) was the first BPDCN-specific drug (2018), with ~70% response in untreated patients and capillary leak syndrome as its signature toxicity. Pivekimab sunirine (CD123 ADC) was approved in 2026 (CADENZA). Venetoclax-based regimens and hyper-CVAD are alternatives, and allogeneic transplant in first remission is the only route to long-term survival. CNS prophylaxis is recommended because of frequent occult CNS involvement.
| Setting | Approach | Guideline |
|---|---|---|
| First line | Tagraxofusp (monitor albumin for capillary leak) or pivekimab sunirine (2026); alternatives hyper-CVAD or venetoclax-based regimens; CNS prophylaxis. | NCCN Category 2A (tagraxofusp) |
| Consolidation | Allogeneic HSCT in first complete remission for eligible patients; autologous transplant in selected cases (Japanese data). | not mapped |
| Relapsed | Alternative CD123 agent, venetoclax combinations, clinical trials; prognosis poor. | not mapped |