Discussing BPDCN with Dr. Muzaffar Qazilbash

Healthtree Foundation recently hosted its first BPDCN webinar with guest speaker Dr. Muzaffar H. Qazilbash, MD. Dr. Qazilbash is a physician and professor in the Department of Stem Cell Transplantation and Cellular Therapy at the University of Texas MD Anderson Cancer Center.

During the webinar, Dr. Qazilbash discussed some of the most common questions he is asked about BPDCN, including:

• Diagnosis
• Risk factors
• Treatment

What is BPDCN?

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive blood cancer. It is most often diagnosed in people aged 60 and up, but it is also diagnosed in children.  Research suggests that it is more common in men than in women. However, because BPDCN is rare, these statistics may change as more information becomes available.  

Diagnosing BPDCN

Like many other rare diseases, a limited understanding of BPDCN can lead to a delay in accurate diagnosis. Dr. Q described BPDCN as an “extremely rare form of acute leukemia” that is now much better understood and characterized than it was in the past

Most people diagnosed with BPDCN have skin lesions. These lesions can vary in size and color and can be found anywhere on the body.  Due to their appearance, they can be mistaken for rashes, bruises, or other skin conditions. 

Read more about how BPDCN is diagnosed.

What are the risk factors?

There are no known environmental exposures or inherited traits that cause BPDCN. 

One of the most common genetic mutations found in people with BPDCN occurs in the TET2 gene, which acts as a tumor suppressor. When this gene mutates, its normal function is impaired, making it less effective in protecting cells from developing into cancer. A recent study suggests that UV damage from the sun could affect the genetic changes to the TET2 gene and the development of BPDCN, but research is ongoing. 

Treatment planning for BPDCN

Once a BPDCN diagnosis has been established, providers will look at individual factors to determine the best treatment. 

Dr. Qazilbash shared that he focuses on the following key things when treating people with BPDCN:

Medical Fitness. Dr. Qazilbash explained that before treatment begins, he needs to evaluate a patient’s general health. This is to determine the best type of treatment for that individual patient. Most patients that Dr. Qazilbash sees are medically fit for the typical BPDCN treatment regimen. In some cases, when a person is much older and has a lot of pre-existing health problems, an aggressive treatment may not be the best option. 

Induction Therapy. Treatment usually begins with induction therapy, which is the first treatment aimed at reducing or eliminating cancer cells in the body. For BPDCN, this is typically chemotherapy. The specific regimen depends on the patient’s age, medical fitness, and whether the cancer has spread to the central nervous system.

Targeted and Novel Therapies. A major advance in BPDCN treatment has been the development of targeted therapies such as tagraxofusp (Elzonris). This treatment specifically targets CD123, a protein commonly found on BPDCN cells. Tagraxofusp has become a standard frontline treatment for many patients. Other treatment options may include traditional chemotherapy combinations similar to those used for acute leukemia. Receiving treatment through a clinical trial studying new agents and combinations is also an option

Stem Cell Transplantation. If the initial therapy works well to control the BPDCN, a stem cell transplant may be recommended to help achieve a long-term remission.

Read more about treatments for BPDCN.

Surveillance. Once remission is achieved, Dr. Qazilbash evaluates what additional treatments are needed to make sure that the patient continues to do well. During this time, your provider will continue to monitor your health and progress so that if there is any risk of the cancer coming back, it will be discovered early.  

When treatment is completed, your provider will create a follow-up plan to make sure that the patient is doing well in remission, and if there is any risk of recurrence, it can be discovered early.  

As awareness and research around BPDCN continue to grow, earlier and more accurate diagnoses are becoming possible. An increased understanding of the signs, symptoms, and genetic features helps providers identify BPDCN sooner, giving patients the best chance to begin appropriate treatment and improve their outcomes. 

Watch the full webinar and hear more from Dr. Qazilbash. 

Healthtree is committed to providing accurate information and resources to people living with blood cancer. You can contribute to this mission by sharing your experience with blood cancer through simple surveys and questionnaires. 

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