Blastic plasmacytoid dendritic cell neoplasm (BPDCN): 7 things to know about this rare leukemia

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a leukemia so rare that it affects only between 500 and 1,000 people in the U.S. each year. It is usually found in males in their late 60s or early 70s, but it can occur in people of any age or gender.

BPDCN was originally thought to be a type of lymphoma, but it’s now considered a subtype of leukemia. It is frequently misdiagnosed because it combines features of leukemia, lymphoma and skin cancer. It can also look like many different things, including melanoma and cutaneous T cell lymphoma.

So, what sets BPDCN apart from other types of leukemia, and what treatments are available? Here’s what to know about this rare and aggressive disease.

How does BPDCN differ from other types of leukemia?

BPDCN develops in our plasmacytoid dendritic cells, which are part of our immune system. Though they make up only a very small portion of our circulating immune cells, plasmacytoid dendritic cells are usually found in the skin, bone marrow, blood, lymph nodes and spleen.

Unlike other blood-based cancers, BPDCN is often found in other locations, which is one of its distinguishing characteristics. It most frequently appears in the skin. It can also appear in the bone marrow, lymph nodes and central nervous system.

About 20% to 30% of the time, it occurs alongside another cancer, such as chronic myelomonocytic leukemia (CMML), myelodysplastic syndrome (MDS) or myeloproliferative neoplasms (MPN). There are indications it could be connected to sun damage, too.

What are the symptoms of BPDCN?

Since BPDCN affects almost every part of the body, its symptoms can be difficult to recognize. But BPDCN is primarily a visual disease, which means you can often see its symptoms. More than 90% of BPDCN patients will have skin lesions at some point.

These lesions usually appear on the arms, legs, face and neck — places that are exposed to ultraviolet light — but they can show up anywhere on the body. Often, they have a purple cast to them, and they range from small, flat discolored spots to large, raised, plaque-like lesions.

BPDCN may also cause swollen lymph nodes and a swollen spleen that can lead to stomach pain.

How is BPDCN diagnosed?

Diagnosing BPDCN often involves ruling out many other conditions first, such as skin infections, autoimmune disorders, side effects of certain drugs, and even other blood disorders, such as leukemia cutis and Sweet syndrome.

To do this, our BPDCN specialists at UT MD Anderson evaluate you in the clinic. First, we inspect your skin for any lesions and determine what percentage of its surface is affected by them. Then, we order a full-body scan to check for lymph node involvement and any other organs that might be affected.

Next, we perform a bone marrow biopsy and a flow cytometry test to check for certain features, including a tumor protein called CD123 that is present in every single case of BPDCN. Finally, we evaluate your cognitive function for central nervous system disease. We may also do genetic testing or next-generation sequencing to further refine your diagnosis.

How is BPDCN treated?

Until recently, there was no standard treatment protocol for BPDCN. We just borrowed chemotherapies used to treat other diseases, like leukemia and lymphoma.

Now, we have two targeted therapy drugs that are specifically designed to treat BPDCN. UT MD Anderson helped pioneer both of these BPDCN drugs. They are:

1. Tagraxofusp (previously known as DT-IL3 or SL-401)
2. Pivekemab sunirine (previously known as IMGN632)

Both act as “Trojan horses,” which means that they wait until they’ve attached to the CD123 protein on the surface of the cancer cells before releasing their deadly payload. The Food and Drug Administration (FDA) approved tagraxofusp for the first-line treatment of BPDCN in December 2018. Pivekemab was just recently approved in May 2026.

If you’re a good candidate for one of these drugs, we’ll likely recommend that first — possibly through a clinical trial. If you respond well and you’re eligible, we may also recommend a stem cell transplant, which is still an integral part of most leukemia treatment plans.

If your central nervous system is involved, though, you’ll probably need intrathecal chemotherapy, too, because neither of those drugs can cross the blood-brain barrier.

And, if you’re not a good candidate for one of those drugs, we’ll recommend something else, based on your unique situation.

What is the life expectancy for someone with BPDCN?

When I first joined UT MD Anderson almost 20 years ago, the overall life expectancy after a BPDCN diagnosis was only about eight months. Today, thanks to these first-in-their-class targeted therapies, we’ve essentially doubled that to around 16 months.

What is the prognosis for someone with BPDCN?

It’s a deadly disease, but we’re making a lot of progress. We didn’t have any treatment options specifically for this disease before. Now we do, so there’s a lot of hope.

And, for some patients, a stem cell transplant is still a curative option. We have a long way to go yet, but a few survivors have actually reached their 10-year milestone after diagnosis.

What's the one thing you want people to know about BPDCN?

Targeted therapies have revolutionized our treatment of this disease. So, we have options now that we’ve never had before. The next step is combining them with other agents — such as chemotherapies, steroids and CAR T cell therapy — to achieve more lasting results.

I think combination therapies are going to be the future for treating BPDCN. Single therapies are just the start.

Naveen Pemmaraju, M.D., is a leukemia specialist at UT MD Anderson with a particular interest in BPDCN.

Request an appointment at UT MD Anderson online or call 1-877-632-6789.