Blastic Plasmacytoid Dendritic Cell Neoplasm
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive blood cancer that develops from plasmacytoid dendritic cells, a type of immune cell that normally helps the body recognize viruses and fight infection.
In BPDCN, these cells become abnormal and grow out of control. The cancer can affect several parts of the body, including the skin, bone marrow and blood, and may also spread to lymph nodes, the spleen or other organs. In many patients, the disease first appears in the skin as purple or bruise-like spots or nodules.
BPDCN most often affects older adults. Because its symptoms can resemble those of other blood cancers, diagnosing the disease can be challenging, and specialized testing is usually needed to confirm the diagnosis and determine the extent of disease.
What causes blastic plasmacytoid dendritic cell neoplasm?
BPDCN develops when plasmacytoid dendritic cells acquire genetic changes that allow them to grow and survive longer than normal cells. As these abnormal cells multiply, they can accumulate in tissues such as the skin, bone marrow or lymph nodes.
Who is at risk?
BPDCN is extremely rare, with only a few hundred cases diagnosed in the United States each year. Doctors have identified certain patterns among people diagnosed with BPDCN:
- Age: Most patients are older adults, typically in their 60s or 70s.
- Sex: The disease occurs more often in men than in women.
Because BPDCN is uncommon, evaluation at a center with experience treating rare blood cancers can help ensure an accurate diagnosis and access to specialized treatment options.
Is BPDCN leukemia or lymphoma?
BPDCN has features of both leukemia and lymphoma. The cancer develops from immune cells that normally circulate in the blood but can also be collected in tissues such as the skin, lymph nodes and bone marrow.
Blastic Plasmacytoid Dendritic Cell Neoplasm Symptoms
Symptoms of BPDCN can vary from person to person and may involve the skin, blood or bone marrow. These may include:
- Skin changes (often the first sign): purple, red, or bruise-like spots or raised bumps that can appear anywhere on the body
- Fatigue or weakness
- Frequent infections
- Easy bruising or bleeding
- Swollen lymph nodes
- Fever or night sweats
- Unexplained weight loss
- Abdominal discomfort caused by an enlarged liver or spleen
What does this condition feel like?
Many patients first notice skin spots that resemble bruises but do not fade. Over time, these spots may increase in number or size.
If the bone marrow becomes involved, symptoms related to low blood counts may develop, such as fatigue, shortness of breath, infections or easy bruising.
Because these symptoms may resemble other conditions, BPDCN may not always be recognized immediately. Persistent or unexplained symptoms should be evaluated by a doctor.
What conditions can look similar?
BPDCN can resemble several other blood cancers or skin conditions. For that reason, doctors rely on specialized laboratory testing to confirm the diagnosis.
Conditions that may appear similar include:
- Other types of leukemia
- Certain lymphomas
- Leukemia cutis (a rare condition where cancerous white blood cells move into the skin)
- Other rare blood cancers affecting the skin
Accurate diagnosis is important because treatment approaches may differ for these conditions.
When to see a doctor
You should talk to your doctor if you notice:
- Unexplained purple or bruise-like skin lesions
- Persistent fatigue or weakness
- Easy bruising or bleeding
- Swollen lymph nodes
- Fever, night sweats or unexplained weight loss
Early evaluation can help determine the cause of symptoms and guide appropriate care.
Survival and prognosis
BPDCN is considered an aggressive disease. However, outcomes have improved in recent years as new therapies have become available.
Prognosis varies depending on several factors, including:
- A patient’s age and overall health
- How much of the body is affected
- How well the disease responds to treatment
Your doctor can help explain what these factors may mean in your individual situation.
Blastic Plasmacytoid Dendritic Cell Neoplasm Diagnosis
Diagnosing BPDCN usually requires several tests because the disease can resemble other cancers. One important step is identifying markers on the cancer cells, including CD123, which helps doctors distinguish BPDCN from other blood cancers.
Doctors may use several tests to confirm the diagnosis and understand how much of the body is affected.
- Skin biopsy: If skin lesions are present, doctors may remove a small sample of tissue and examine it under a microscope.
- Bone marrow biopsy: A bone marrow sample helps determine whether the disease involves the bone marrow or blood.
- Immunophenotyping and laboratory testing: Specialized laboratory tests identify proteins found on BPDCN cells, including markers such as CD4, CD56 and CD123. These markers help doctors confirm the diagnosis.
- Genetic and molecular testing: These tests may identify genetic changes that provide additional information about the disease and help guide treatment decisions.
Because BPDCN can be difficult to diagnose, results are often reviewed by specialists experienced in blood cancers.
Blastic Plasmacytoid Dendritic Cell Neoplasm Treatment
Treatment for BPDCN depends on several factors, including a patient’s overall health, how much of the body is affected and how the disease responds to therapy.
Treatment options may include targeted therapy, chemotherapy and stem cell transplantation.
Targeted therapy
Targeted therapy drugs are designed to stop or slow the growth or spread of cancer. This happens on a cellular level. Cancer cells need specific molecules (often in the form of proteins) to survive, multiply and spread. These molecules are usually made by the genes that cause cancer, as well as the cells themselves. Targeted therapies are designed to interfere with, or target, these molecules or the cancer-causing genes that create them.
Patients with BPDCN may receive targeted therapy through intravenous (IV) infusion. Treatment is typically given in cycles over several weeks. Side effects depend on the exact drug the patient receives and may include fatigue, nausea, swelling, low blood pressure or changes in liver function.
Learn more about targeted therapy
Chemotherapy
Chemotherapy drugs kill cancer cells, control their growth or relieve disease-related symptoms. Chemotherapy may involve a single drug or a combination of two or more drugs, depending on the type of cancer and how fast it is growing.
Patients usually receive chemotherapy through intravenous (IV) infusions. Treatment is typically given in cycles over several weeks. Side effects depend on the exact drug the patient receives and may include hair loss, nausea, fatigue, infections and low blood counts.
Stem cell transplantation
A stem cell transplant (also known as a bone marrow transplant) is a procedure that replaces cancerous bone marrow with new, healthy bone marrow stem cells. Depending on the cancer, the healthy stem cells can come from a donor or from the patient. Stem cell transplants are usually given after an intense round of chemotherapy that kills the patient’s existing bone marrow cells. Patients usually must stay in the hospital for three to four weeks after the transplant.
Learn more about stem cell transplantation
Clinical trials at UT MD Anderson
Clinical trials play an important role in improving treatment for BPDCN and helping researchers better understand the disease.
UT MD Anderson is one of the leading centers studying BPDCN, with physicians and scientists actively conducting clinical trials and publishing research to improve treatment approaches.
Participating in a clinical trial may give some patients access to promising new therapies that are not yet widely available. Your care team can help determine whether a clinical trial may be appropriate for your situation.
To see currently available studies, visit the UT MD Anderson clinical trials search page.
Why choose UT MD Anderson for your blastic plasmacytoid dendritic cell neoplasm treatment?
Choosing where to receive care is an important decision after a diagnosis of BPDCN. Experience in diagnosing and treating this disease can make a meaningful difference.
At UT MD Anderson, patients are treated by physicians who specialize in leukemia and other hematologic malignancies. Specialists use advanced testing to confirm the diagnosis and determine the extent of disease.
Care is delivered through multidisciplinary teams that may include hematologic oncologists, dermatologists, pathologists and transplant specialists. These experts work together to develop treatment plans tailored to each patient.
UT MD Anderson physicians and scientists are also advancing research in BPDCN through clinical studies and peer-reviewed publications that help improve treatment approaches.
Patients have access to a wide range of supportive services, including nutrition counseling, social work support, integrative medicine and survivorship programs.
Leukemia specialists: How advances in measurable residual disease (MRD) are improving cancer care
Measurable residual disease (MRD) is a monitoring tool that physicians use to assess the remission status of patients with leukemias, lymphomas, myelomas and other blood disorders.
Formerly known as “minimal residual disease,” this term describes a situation in which cancer cells are still present in the body after treatment, yet undetectable using current testing methods.
Two of our leukemia specialists, Naveen Pemmaraju, M.D., and Fadi Haddad, M.D., explored this subject in a recent Cancerwise podcast. Here are three highlights from that discussion.
Next-generation sequencing can now reveal ‘a needle in a haystack’
Patients with blood cancers used to be monitored for relapse using:
- Flow cytometry, which reveals disease through certain patterns of refracted laser light, and
- Polymerase chain reaction (PCR) tests, which are highly sensitive molecular tests that detect specific genetic material from cancer cells
But sometimes, patients would seem to be cured based on these tests, only to relapse some months later. That got doctors to questioning why. Here’s the conclusion ours came to: even if some patients appeared to have no residual disease, it was only because we weren’t able to detect it yet.
One possible solution? Next-generation sequencing.
“With next-generation sequencing, we can pick out a single leukemia cell from a sea of a million normal ones,” explains Haddad. “This is a very, very minimal level of disease that would’ve previously gone unnoticed — like a needle in a haystack. This new test can help us detect any little seed left behind so we can completely eradicate the cancer. This marks a real paradigm shift.”
5 emerging therapies presented at ASCO 2025
Every year, the American Society of Clinical Oncology Annual Meeting provides a platform for sharing exciting new cancer therapies. In 2025, MD Anderson clinicians and researchers will present more than 120 studies. Five stand out for their potential to change the way cancers are treated.
They include therapies for thyroid cancer, colorectal cancer and a rare type of leukemia. The studies represent a variety of first-in-class approaches for treating cancer, from a lipid nanoparticle-encapsulated mRNA to a new oral small molecule inhibitor.
1. Thyroid cancer combination therapy
Patients with a type of anaplastic thyroid cancer in which BRAF V600E is mutated tend to have a poor prognosis because they are often diagnosed when the disease is already advanced and can’t be removed with surgery.
In this Phase II trial, presented by Mark Zafereo, M.D, professor of Head and Neck Surgery, patients with Stage IV BRAF V600E-mutated anaplastic thyroid cancer were given neoadjuvant pembrolizumab in combination with dabrafenib and trametinib — a combination called DTP. Afterward, they had surgery to remove any remaining cancer, and on average, this was far more successful than historic averages in patients who hadn’t been treated first with DTP. Two-thirds of patients had no residual anaplastic thyroid cancer, and these patients had an overall two-year survival rate of 69%.
These results are a strong indication that DTP treatment before surgery enables a higher rate of successful surgical resection.
2. New BRAF-mutated metastatic colorectal cancer first-line treatment
BRAF V600E–mutated metastatic colorectal cancer is an aggressive subtype with a poor prognosis. A Phase III clinical trial, led at MD Anderson by co-principal investigator Scott Kopetz, M.D., Ph.D., professor of Gastrointestinal Medical Oncology and associate vice president of Translational Integration, is testing whether encorafenib plus cetuximab, with or without chemotherapy, will be a better first-line treatment.
Earlier results showed a 60.9% overall response rate with the three-drug combination, compared with 40% for the current standard-of-care treatment. These findings led to accelerated Food and Drug Administration (FDA) approval of this combination therapy for BRAF V600E–mutated metastatic colorectal cancer, including as first-line therapy. The latest results take that one step further and show significantly longer progression-free and overall survival for patients treated with the three-drug combination, compared with those who received the current standard of care.
Personalized treatment helps retired physician overcome dual cancer diagnoses
As a medical student at Baylor College of Medicine, Steve Meltzer traveled past MD Anderson many times. However, he never thought he would one day be a patient here.
In October 2020, just six weeks before he planned to retire from his long career as a physician, he was simultaneously diagnosed with both T-cell acute lymphoblastic leukemia (T-ALL) and blastic plasmacytoid dendritic cell neoplasm (BPDCN). BPDCN is a rare and aggressive blood cancer that often transforms into acute leukemia. The disease impacts approximately 500 to 1,000 people in the U.S. each year. T-ALL is also an acute leukemia that begins in the bone marrow and lymph nodes and can spread to other organs.
For Steve, the diagnosis came as a surprise, and it took several tests and doctors’ appointments to explain a mysterious mass on his clavicle.
An ear, nose and throat doctor had encouraged him to get a biopsy of the mass, but that testing didn’t find anything. “My white blood cell count was very low, but there was no indication of cancer,” Steve recalls. “Later, I went to a hematologist to repeat more blood work and do a bone marrow aspiration, which also came back negative.”
By this time, the mass had grown slightly. His doctors were stumped. As a physician himself, so was Steve.
A dual diagnosis: BPDCN and T-cell acute lymphoblastic leukemia
During a routine skin screening with his dermatologist, she saw the mass and insisted he get a third opinion. This time a surgeon removed the mass, examined it and determined it was BPDCN and T-ALL.
“It’s hard to know when the mass really started. Doctors were stumped until the mass was finally removed,” Steve says. “This type of cancer is so rare that there are few doctors who treat it, but my hematologist found Dr. Pemmaraju and said go to MD Anderson.” Meltzer met with Naveen Pemmaraju, M.D., a leukemia expert specializing in BPDCN.
BPDCN is often misdiagnosed since it is so rare and shares symptoms of other blood cancers.
“I’m glad he was able to get to us when he did because we were able to assess him and get a treatment plan going almost immediately,” Pemmaraju says. “We initially wanted to enroll him in a clinical trial that was ongoing at the time; however, because he also had heart disease, we had to discuss alternatives.”
Doctors personalize BPDCN and T-cell acute lymphoblastic leukemia treatment
Pemmaraju worked with oncocardiologist Elie Mouhayar, M.D., and stem cell transplant specialist Muzaffar Qazilbash, M.D., to examine Steve’s case. They determined chemotherapy followed by stem cell transplant was the best route for him and would ensure his heart would withstand treatment.
The chemotherapy he received was an innovative hybrid regimen incorporating systemic alternating multi-agent chemotherapy and central nervous system prophylactic chemotherapy developed by Pemmaraju and Hagop Kantarjian, M.D., for Steve’s unique case of extremely rare, life-threatening dual cancers and heart disease. The personalized, innovative regimen was based on chemotherapy principles of multi-agent delivery that Kantarjian originally described and pioneered over the past several decades. The chemotherapy and stem cell transplant were both successful.
Gratitude for the experts
Four years later, both cancers are in remission, and Steve has tremendous gratitude for all the doctors who played a role in determining his diagnosis and treating him successfully.
“I’ve been able to enjoy retirement with my wife, watch television together for what feels like the first time in decades, work on my old jalopies, and spend time with our friends and family,” Steve says. “I relinquished a lot of control during my treatment at MD Anderson, which is rare for me as a physician. I trusted the experts, and they did not let me down.”
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Featured Podcast:
Tracking leukemia with minimal residual disease (MRD)
Naveen Pemmaraju, M.D., and Fadi Haddad, M.D., explain how minimal residual disease, or MRD, is helping doctors monitor leukemia more precisely and personalize treatment.