BPDCN Prevalence, Patient Profile, and Work-Up

Gary Schiller, MD: Welcome to this OncLive® NewsNetwork® presentation, “Targeted Therapy in Blastic Plasmacytoid Dendritic Cell Neoplasm.” We’re going to discuss the advanced treatment of BPDCN [blastic plasmacytoid dendritic cell neoplasm]. I’m Gary Schiller, and I’m the director of the hematologic malignancy stem cell transplant program at the David Geffen School of Medicine at UCLA Medical Center in Los Angeles, California. Today I am joined by my colleague Dr Salman Fazal, from West Penn Hospital in Pittsburgh, Pennsylvania.

Salman Fazal, MD: I’m the director of transplant at the Allegheny Health Network in Pittsburgh, Pennsylvania, and specialize in myeloid malignancies.

Gary Schiller, MD: We’re going to discuss blastic plasmacytoid dendritic cell neoplasm. This is a myeloid malignancy. It is not common. It’s been considered quite rare, and it was underdiagnosed in the past. It will take more data and a few more years of patient presentation to get an accurate assessment as to how common this myeloid neoplasm is. A typical patient profile is the reason that the disease is underdiagnosed. Often the patients are older, although there is no specific age distribution, and they present to a dermatologist with skin lesions. It is very common that patients will present with a variety of skin lesions, typically papular ecchymotic skin lesions on the trunk but also on the extremities. They’re often palpable, can sometimes be on the face, and can sometimes be macular. Given time, patients will go on to develop pancytopenia as this neoplasm will invade the marrow and produce hypoproliferation of normal marrow elements.The typical work-up depends on where the patient presents. If a patient presents in a dermatologist’s office, the skin biopsy may lead to a diagnosis. If the patient’s pancytopenia is associated with complications such as neutropenic fever or thrombocytopenia, that patient might present in an emergency department, in an internal medicine ward, or hopefully on a hematology service, and a bone marrow might be the way the diagnosis is made. The most important thing in making a diagnosis is histopathologic confirmation and working with an hematopathologist, because it’s a tricky diagnosis. It’s not easy, and a hematopathologist needs to be involved from the skin biopsy all the way through the bone marrow biopsy. Otherwise it will be underdiagnosed or misdiagnosed as some other primary dermatologic neoplasm.

Transcript Edited for Clarity