Blastic plasmacytoid dendritic cell neoplasm: Evolving insights in an aggressive hematopoietic malignancy with a predilection of skin involvement

Shweta Gera, Mhair S. Dekmezian, Madeleine Duvic, Jaime A. Tschen, Francisco Vega, Jeong Hee Cho-Vega

Research output: Contribution to journalReview article

8 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)244-251
Number of pages8
JournalAmerican Journal of Dermatopathology
Volume36
Issue number3
DOIs
StatePublished - Jan 1 2014
Externally publishedYes

Fingerprint

Hematologic Neoplasms
Dendritic Cells
Skin
Neoplasms
Acute Myeloid Leukemia
Molecular Biology
Lymphoma
Leukemia
Biopsy

Keywords

  • BCL11A
  • Blastic natural killer cell lymphoma
  • Blastic plasmacytoid dendritic cell neoplasm
  • CD123
  • TCL-1

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Dermatology

Cite this

Blastic plasmacytoid dendritic cell neoplasm : Evolving insights in an aggressive hematopoietic malignancy with a predilection of skin involvement. / Gera, Shweta; Dekmezian, Mhair S.; Duvic, Madeleine; Tschen, Jaime A.; Vega, Francisco; Cho-Vega, Jeong Hee.

In: American Journal of Dermatopathology, Vol. 36, No. 3, 01.01.2014, p. 244-251.

Research output: Contribution to journalReview article

Gera, Shweta ; Dekmezian, Mhair S. ; Duvic, Madeleine ; Tschen, Jaime A. ; Vega, Francisco ; Cho-Vega, Jeong Hee. / Blastic plasmacytoid dendritic cell neoplasm : Evolving insights in an aggressive hematopoietic malignancy with a predilection of skin involvement. In: American Journal of Dermatopathology. 2014 ; Vol. 36, No. 3. pp. 244-251.
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