Dr. Lane on Identification of BPDCN

Andrew A. Lane, MD, PhD, physician, assistant professor of medicine, Harvard Medical School, Dana-Farber Cancer Institute, discusses the identification of a rare blood disorder called blastic plasmacytoid dendritic cell neoplasm (BPDCN).

This disease has been one without a home, or proper identification, for the last 20 to 30 years, Lane explains, adding that it is most recently thought to be related to acute myeloid leukemia (AML). BPDCN presents in the bone marrow as well as in lymph nodes. Ninety percent or more of patients, he adds, also have skin lesions. Until 2008, the disease was not identified, was lumped into various disease groups, and was treated by leukemia and lymphoma specialists with a number of chemotherapy regimens that did not perform well. In this aggressive disease, the median survival remains to be less than 1 year.

Now named BPDCN, more research has been conducted in this specific area; however, the estimated number of patients with the disease is still unclear. It is related to myeloid diseases and 20% to 25% of patients have a myelodyplastic syndrome (MDS)-like disorder. Moreover, when a patient with MDS transforms to acute leukemia, Lane questions whether they could actually have BPDCN, because that could also arise out of MDS.