Moving Toward A Deeper Understanding of the Characteristics of BPDCN

Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare and aggressive blood cancer that can be challenging to diagnose. There are many symptoms associated with BPDCN that impact the skin, lymph nodes, and central nervous system (CNS), which includes the brain and spinal cord.  

Read about the symptoms of BPDCN.  

Diagnosing BPDCN accurately early can have a significant impact on overall outcomes. The sooner a provider has precisely identified the cancer, the sooner treatment can begin. As with many other blood cancers, some treatments are not available to patients once their cancer has progressed to a certain point. So being diagnosed early can mean more treatment options for patients.   

Read about diagnosing BPDCN.  

A recent study evaluated registry data on 257 patients with BPDCN to better understand how thorough characterization of BPDCN supports better disease classification and treatment decisions.  

Researchers evaluated cells through immunophenotyping and/or immunohistochemistry. These are specialized laboratory tests to examine the surface or inside of tissue after a biopsy for evidence of disease. They used this data to establish the key signs and symptoms of BPDCN, including cell markers.   

Key diagnostic markers and clinical features identified in BPDCN

Skin Involvement 

The most common symptom of BPDCN is skin lesions. They can appear as nodules, plaques, or bruise-like patches and are typically red, purple, or brown in color. These lesions can be anywhere on the body.  They are often itchy or painful but in some cases are asymptomatic. Of the participants studied, skin involvement occurred in 81% of people.  

Lymph Nodes 

BPDCN can cause the lymph nodes to become enlarged and swollen, often felt as lumps under the skin. BPDCN was found in the lymph nodes in 49% of people studied. 

CNS Involvement

BPDCN can also spread to other organs and can impact the central nervous system, leading to neurological symptoms that differ in severity.  CNS involvement was recorded in 29% of people from the registries. 

Cell Markers

Doctors can determine whether a person has BPDCN by looking for a special combination of cell markers. Information about genetic expression is critical for the development of targeted therapies, which are treatments directed at certain markers while reducing the impact on healthy cells. 

BPDCN usually shows a pattern that includes three key markers: CD123, CD4, and CD56.This unique set of markers helps doctors distinguish BPDCN from other types of blood cancer and make a clear and accurate diagnosis. There was positive expression of CD123, CD4, CD56, and TCL1 in most cases. 

What this research means for the future of BPDCN

As the understanding of BPDCN continues to develop, using a structured identification process can improve diagnosis, treatment, and overall outcomes for BPDCN.  

This research can be used in setting standards that help:  

• Confirm diagnosis early by distinguishing BPDCN from other blood cancers that have similar characteristics.

• Improve treatment options by using information to design therapies that target specific markers

To continue reading more articles like this about BPDCN, follow the link below. 

Read More BPDCN News

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Sources:

• Disease presentation and immunophenotype characteristics in 257 patients with BPDCN 
• HealthTree-BPDCN Signs and Symptoms 
• HealthTree-Diagnosing BPDCN 
• Cleveland Clinic-Immunophenotyping