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Blastic Plasmacytoid Dendritic Cell Neoplasm: Evolving Insights in an Aggressive Hematopoietic Malignancy With a Predilection of Skin Involvement

Gera, Shweta MD*; Dekmezian, Mhair S.; Duvic, Madeleine MD; Tschen, Jaime A. MD§; Vega, Francisco MD, PhD; Cho-Vega, Jeong Hee MD, PhD§

The American Journal of Dermatopathology: March 2014 - Volume 36 - Issue 3 - p 244–251
doi: 10.1097/DAD.0b013e318280de99
Review Article

Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) derived from precursors of plasmacytoid dendritic cells is a very rare, unique, and highly aggressive immature hematopoietic malignancy, more frequently occurring among healthy elderly adults. BPDCN can be characterized by a striking predilection for cutaneous involvement, which is often detected incidentally by dermatologists and is difficult to clinically distinguish it from other primary skin lesions and histologically from leukemia/lymphoma cutis. Thus, histological diagnosis of cutaneous biopsies is crucial to correctly classify this entity. Most patients eventually progress to acute myeloid leukemia and are generally not curable. Here, we present 2 cases of classic BPDCN and discuss the origin of tumor and literature-based characteristic clinical and morphological features, evolving immunomarkers, and molecular genetic aspects of this neoplasm.

*Department of Pathology, St. Luke's Roosevelt Hospital, New York, NY

Baylor College of Medicine, Houston, TX

Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, TX

§St. Joseph Dermatopathology, Houston, TX

Department of Hematopathology, University of Texas MD Anderson Cancer Center, Houston, TX.

Reprints: Jeong Hee Cho-Vega, MD, PhD, St. Joseph Dermatopathology, 6909 Greenbrier St, Houston, TX 77005 (e-mail:

The authors declare no conflicts of interest.

© 2014 by Lippincott Williams & Wilkins.