Blastic Plasmacytoid Dendritic Cell Neoplasm

Blastic Plasmacytoid Dendritic Cell Neoplasm

Kaaren K. Reichard, MD

Bone marrow aspirate smear from a patient with blastic plasmacytoid dendritic cell neoplasm shows typical lymphoblast-like cytology. This case also shows pseudopod formation.

Bone marrow core biopsy from a patient with blastic plasmacytoid dendritic cell neoplasm shows marrow effacement by a variably sized blastic neoplasm. Immunophenotypic studies reveal the cell lineage.



  • Blastic plasmacytoid dendritic cell neoplasm (BPDCN)


  • CD4(+)/CD56(+) hematodermic neoplasm

  • Blastic NK-cell lymphoma

  • CD4(+)/CD56(+) lineage-negative malignancy

  • Agranular CD4(+) natural killer-cell leukemia


  • Malignancy of plasmacytoid dendritic cells

  • Neoplasm with typical cutaneous and bone marrow involvement (“hematodermic”)


Environmental Exposure

  • None clearly identified

Infectious Agents

  • No link with Epstein-Barr virus infection

Postulated Normal Cell Counterpart

  • Plasmacytoid dendritic cell

    • a.k.a. interferon-producing cell, dendritic cell type 1 (DC1)

    • a.k.a. (previously) plasmacytoid monocyte, plasmacytoid T cell

    • Produces high amounts of α-interferon

    • Innate immunity

    • Derives from either common lymphoid or myeloid precursor in bone marrow

Some Cases Associated with Myeloid Neoplasia

  • BPDCN and myelodysplasia

  • BPDCN and myelomonocytic leukemia



  • Incidence

    • Exceedingly rare

  • Age

    • Mainly adults

      • Average age range: 60-67 years

    • Children and young adults also

  • Gender

    • Male predominant; M:F ratio = 3-4:1

  • Ethnicity

    • No known ethnic predisposition


  • BPDCN involves multiple tissues

    • Skin

      • > 95% of cases

      • Often initial site of disease

    • Peripheral blood and bone marrow

      • 60-90% of cases

      • May be involved at diagnosis (˜ 30-40%) or at relapse

    • Lymph nodes

      • 30-40%


  • Skin lesions

  • Abnormal CBC

  • Circulating neoplastic cells

  • Cytopenias

  • Blastic bone lesions


  • Options, risks, complications

    • Definitive treatment unclear with so few cases

  • Multiagent chemotherapy

    • Some advocate acute leukemia-type regimen

    • Some advocate high-grade lymphoma-type regimen


  • Aggressive clinical course

  • Median survival: 12-14 months

  • Most cases initially respond to multiagent chemotherapy (> 80%)

  • Subsequent relapse in treatment resistant

  • Rare reports of long-term survival


General Features

  • Skin lesions

  • Splenomegaly (< 20%)

  • Hepatomegaly (< 20%)


Predominant Pattern/Injury Type

  • Infiltrative

Predominant Cell/Compartment Type

  • Dendritic cell

Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Blastic Plasmacytoid Dendritic Cell Neoplasm
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