New blastic plasmacytoid dendritic cell neoplasm (BPDCN) treatment improves outlook for many patients

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare type of acute leukemia that impacts about 500-1,000 people in the U.S. each year. It mainly affects older patients, but can affect people of any age. Blastic plasmacytoid dendritic cell neoplasm springs from a rare immune cell that is usually found in the skin, bone marrow, blood, lymph nodes and spleen. Because it affects almost every part of the body and its name has changed so many times over the past four decades, BPDCN can be difficult to understand, recognize, diagnose and treat. This aggressive disease has had historically few treatment options and a poor prognosis. But a new treatment option — a targeted therapy drug called tagraxofusp — has demonstrated clinical improvement for patients in a recently completed major clinical trial. Naveen Pemmaraju, M.D., led the clinical trial for this new drug. He recently spoke with us about what makes the disease so hard to treat and what the new medication means for patients. Here’s what he had to say. What are the symptoms of blastic plasmacytoid dendritic cell neoplasm? BPDCN affects patients’ skin, bone marrow, lymph nodes and blood. Since it affects almost every single part of the body, blastic plasmacytoid dendritic cell neoplasm symptoms can be difficult to recognize, making the disease difficult to diagnose. Practically every BPDCN patient gets skin lesions. They often appear on, arms, legs, face and neck, but can show up anywhere on the body. In addition, the disease can cause swollen lymph nodes and a swollen spleen that can lead to stomach pain. So it combines features of leukemia, lymphoma and skin...