title: Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rapidly Progressive and Fatal Disease without Aggressive Intervention.
abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive myeloid neoplasm derived from plasmacytoid monocytes. The most common presentation involves cutaneous manifestations, which are often accompanied by bone marrow involvement. The tumor cells reveal an immature blastic appearance and diagnosis is based on the expression of cluster of differentiation (CD)4 and CD56. The literature reports a high relapse rate and poor prognosis when treated with leukemia-type induction chemotherapy alone; however, long-term remission is attainable with allogeneic stem cell transplantation in the first complete remission. Here, we report the dismal course of a patient with BPDCN with cutaneous and bone marrow involvement unable to undergo an aggressive intervention.
authors.affiliation: Resident Physician, Department of Internal Medicine, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS, USA.
authors.forename: Lindsey
authors.initials: L
authors.lastname: Prochaska
authors.affiliation: Hematology/Oncology Fellow, Department of Internal Medicine, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS, USA.
authors.forename: Christopher
authors.initials: C
authors.lastname: Dakhil
authors.affiliation: Pathologist, Department of Pathology and Laboratory Medicine, Kansas City VA Medical Center, 4801 East Linwood Boulevard, Kansas City, MO, USA.
authors.forename: Sharad
authors.initials: S
authors.lastname: Mathur
keywords: BPDCN; blastic plasmacytoid dendritic cell neoplasm; natural killer-cell lymphoma; neoplasm