blastic plasmacytoid dendritic cell neoplasm


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  • Clinical

    • skin lesions
    • regional lymphadenopathy in 20%
    • later marrow and PB involvement
    • can transform into leukemia
    • AML association in 10-20%

  • Histology

    • dermal and subq infiltrate, lacks epidermotropism
    • can be monomorphic or polymorphic
    • blast infiltrate
    • lacks angioinvasion or necrosis

  • Immunophenotype

    • plasmacytoid dendritic cell antigens: CD123, BDCA2/CD303, TCL1, CLA, MxA
    • positive: CD4, CD56, CD123, CD43, CD45RA
    • negative: CD3, MPO, cytotoxic (granzymeB, TIA1, perforin), EBV

  • Molecular

    • lack TCR rearrangement
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