What is Blastic Plasmacytoid Dendritic Cell Neoplasm?

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive type of blood cancer, which primarily affects the skin and then progresses to the bone marrow. It was previously known as blastic natural killer (NK) cell lymphoma or CD4+/CD56+ hematodermic neoplasm. BPDCN is characterized by the presence of tumor cells that resemble plasmacytoid dendritic cells, a type of immune cell. These cells are typically found in the skin, lymph nodes, bone marrow, and peripheral blood. The disease often presents with skin lesions such as nodules, bruise-like patches, or ulcers. Other symptoms may include fever, weight loss, and frequent infections.

Why Do People Get Blastic Plasmacytoid Dendritic Cell Neoplasm?

The exact cause of BPDCN is not known. However, it is believed to be associated with genetic mutations. Some studies have identified mutations in the TET2, ASXL1, and NRAS genes in people with BPDCN. These genes are involved in cell growth and division, and mutations can lead to uncontrolled cell growth, which is a hallmark of cancer. The disease is not considered hereditary and does not seem to run in families. Other risk factors such as environmental exposures or lifestyle factors have not been clearly identified.

Who Gets Blastic Plasmacytoid Dendritic Cell Neoplasm?

1,000 to 1,400 new cases of BPDCN are diagnosed in the United States and Europe combined each year. While BPDCN can occur at any age, but it is more common in older adults. The median age at diagnosis is mid-60s. It is also more common in males than in females. Approximately 75% of BPDCN patients are male. 

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• How Is Blastic Plasmacytoid Dendritic Cell Neoplasm Diagnosed?
• How Long Will I Live With Blastic Plasmacytoid Dendritic Cell Neoplasm?
• What Are The Signs And Symptoms Of Blastic Plasmacytoid Dendritic Cell Neoplasm?
• How Is Blastic Plasmacytoid Dendritic Cell Neoplasm Staged And Classified?
• What Are Treatments For Blastic Plasmacytoid Dendritic Cell Neoplasm?