Blastic plasmacytoid dendritic cell neoplasm (BPDCN) was previously known as natural killer (NK) cell leukemia/lymphoma.
In 2008, as the understanding of the biology and origin of this malignancy has improved, the World Health Organization (WHO) established the term “blastic plasmacytoid dendritic cell neoplasm (BPDCN).”
BPDCN is currently listed as its own category in the 2016 WHO revision. Most often, BPDCN presents with features of both lymphoma and leukemia. The average age at diagnosis is 60 to 70 years, and more men than women are diagnosed with BPDCN.
BPDCN diagnosis and incidence
BPDCN is very often misdiagnosed and underreported. Diagnosing a patient with BPDCN is difficult.
The skin is the most frequently involved site of disease (in 80 percent of cases). However, BPDCN usually progresses with bone marrow involvement and a decrease in red and white blood cells and platelet counts. The lymph nodes and spleen may also be involved. Rashes without these symptoms can also occur.
Common misdiagnoses for BPCDN include non-Hodgkin lymphoma (NHL), acute myeloid leukemia (AML), leukemia cutis (a nonspecific term used for cutaneous (skin) manifestation of any type of leukemia), melanoma (a type of skin cancer), and lupus erythematosus (chronic inflammatory disease that occurs when your body's immune system attacks your own tissues and organs).
Current treatment and outcomes
Tagraxofusp-erzs (Elzonris®) is approved for the treatment of BPDCN in adult and pediatric patients two years and older.
Treatment sometimes includes therapies that are used for AML, acute lymphoblastic leukemia (ALL), or lymphoma.
The length for which a patient responds to these treatments is usually short. After a relapse, second remissions with conventional chemotherapy are difficult to achieve. Allogeneic hematopoietic stem cell transplant (allo-HCT), especially if offered during the first remission, may result in longer remissions. The current recommendation is for BPDCN patients to be evaluated for an allo-HCT as soon as possible and to begin searching for a donor.
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Research and evolving novel treatments
The length of remission from current treatments is unknown. Patients should consider clinical trials as treatment options. Currently, there are a few ongoing clinical trials for BPDCN.
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