Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare type of blood cancer.
Skin changes are often the first and most common signs of BPDCN. They may look like bruises, raised patches, or lumps and can appear in one area or many places on your skin. BPDCN may also affect the bone marrow, blood, lymph nodes, organs, and other parts of the body.
BPDCN has usually been treated with medicines such as chemotherapy and targeted therapies, and some people may also receive a stem cell transplant. In May 2026, the FDA approved the first medicine in its class to treat BPDCN: Decnupaz (pivekimab sunirine). Decnupaz may be used as a first treatment for people with newly diagnosed BPDCN. It can also be used to treat BPDCN that has come back or did not respond to previous treatments.
Keep reading to learn how Decnupaz works, how it was studied, what benefits were seen, and how it is given.
How Does Decnupaz Work for BPDCN?
Decnupaz belongs to a class of targeted cancer medicines called antibody-drug conjugates (ADCs). ADCs contain two parts, a protein called an antibody and an anticancer drug. The antibody acts as the guide by seeking out and attaching to a specific target called CD123. CD123 is a protein found on the surface of BPDCN cancer cells.
Once the antibody part of Decnupaz attaches to CD123, the cancer cell takes the medicine inside. Decnupaz then releases an anticancer drug called FGN849. FGN849 damages the cancer cell’s DNA. This damage can cause the cancer cell to die.
Decnupaz can help bring BPDCN into remission, meaning signs of the cancer go away. But remission does not always mean the cancer is gone for good. That’s why some people undergo a stem cell transplant for BPDCN. A stem cell transplant can help some people stay alive and in remission longer, especially when it is done after reaching complete remission.
In the main clinical study, some people were able to receive a stem cell transplant after reaching remission with Decnupaz. You may hear this or other medicines being described as a “bridge” to stem cell transplant, but it is not always a bridge. Not everyone can safely get a stem cell transplant, and some people may have already had a stem cell transplant in the past before being treated with Decnupaz.
Your health care provider can discuss your options with you to treat your BPDCN.
How Do I Use Decnupaz?
Decnupaz is given into a vein through an intravenous (IV) line once every three weeks. Your health care provider will prescribe your dose based on your body weight.
Decnupaz infusions usually take about 15-30 minutes. The first infusion generally takes longer, and you will also need monitoring for many hours afterward to watch for reactions. If you tolerate it well, later infusions and the need for monitoring may be shorter.
You may need to take medicine(s) the day before your infusion and 30-60 minutes before each Decnupaz infusion. These are premedicines, which help lower the chance of certain reactions. Follow the instructions from your health care provider about when to take these medicines.
Decnupaz treatment may continue until your BPDCN gets worse or your side effects make it unsafe to continue. If you have severe side effects, your health care provider may delay your next infusion, adjust your dose, or stop Decnupaz altogether. You will also need to have blood tests during treatment to check for liver problems and other possible side effects.
To learn more, see our article about managing Decnupaz side effects and interactions.
How Was Decnupaz Studied?
Decnupaz was studied for treating BPDCN in a clinical trial called CADENZA. The study included people who fit into one of two groups:
- Adults with newly diagnosed BPDCN that had not yet been treated — in other words, Decnupaz would be the first treatment for their cancer
- Adults with BPDCN that had returned (relapsed) or did not respond to previous treatments (refractory)
People could not join the study if they had certain treatment options still available, active or uncontrolled BPDCN involving the brain or spinal cord (central nervous system), a history of certain serious liver or fluid-related problems, or received certain cancer treatments in the recent past. People also needed to have recovered from short-term side effects of prior cancer treatment.
During the study, all participants received Decnupaz as an IV infusion once every three weeks.
To test how well Decnupaz worked, the researchers looked at the percentage of people who entered complete remission, meaning they had no signs of active BPDCN. People could still be counted as in remission if they had a remaining skin change that was not active cancer.
The table below summarizes some key details about the participants in each group at the start of the study.
| Newly Diagnosed BPDCN | Relapsed or Refractory BPDCN | |
|---|---|---|
| Number of people | 33 | 51 |
| Median (middle) age | 73 years | 69 years |
| Age range | 48-84 years | 19-85 years |
| Sex | 82% male, 18% female | 82% male, 18% female |
| Race | 82% White, 15% unreported, 3% Black | 82% White, 12% unreported, 4% Black, 2% Asian |
| Ethnicity | 85% not Hispanic or Latino, 12% Hispanic or Latino, and 3% unknown | 75% not Hispanic or Latino, 16% Hispanic or Latino, 10% unknown |
| Previous stem cell transplant | — | 31% |
| BPDCN in skin | 94% | 67% |
| BPDCN in bone marrow | 49% | 47% |
| BPDCN in blood | 9% | 20% |
| BPDCN in lymph nodes | 36% | 35% |
| BPDCN in internal organs | None | 6% |
What Benefits Were Seen With Decnupaz in the Study?
Decnupaz led to remission in some adults from each group. Response rates were higher in people with newly diagnosed BPDCN compared with people whose cancer had returned (relapsed) or did not respond to previous treatments (refractory).
Newly diagnosed BPDCN
- Among the 33 people with newly diagnosed BPDCN, 23 people (about 70%) reached complete remission or clinical complete remission.
- This included 16 people who reached complete remission and seven people who reached clinical complete remission.
Complete remission means signs of active BPDCN could no longer be detected. Clinical complete remission is similar, but a person could still have skin changes that were not considered active cancer.
The median time to reach one of these responses was 1.8 months. Among people who responded, the median duration of remission (how long it lasted) was 9.7 months.
After treatment with Decnupaz, 13 of the 33 people in this group (about 39%) went on to receive a stem cell transplant.
Relapsed or refractory BPDCN
- Among the 51 people whose BPDCN had returned or had not responded to previous treatment, eight people (about 16%) reached complete remission or clinical complete remission.
- This included seven people who reached complete remission and one person who reached clinical complete remission.
The median time to reach one of these responses was 1.7 months. Among people who responded, the median duration of complete remission or clinical complete remission was 9.2 months.
After treatment with Decnupaz, 6 of the 51 people in this group (about 12%) went on to receive a stem cell transplant.
Your results may be different from what was seen in the study. Note that this study did not include a comparison group, so this study does not show how Decnupaz compares with another treatment for BPDCN. Your health care provider can help decide whether Decnupaz is an option for you and help you understand what to expect.
