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  • Title: Expanding the Immunophenotypic Spectrum of Neoplastic and Reactive Plasmacytoid Dendritic Cells.
    Author: Wu SJ, Sadigh S, Lane AA, Pinkus GS.
    Journal: Am J Clin Pathol; 2023 May 02; 159(5):455-463. PubMed ID: 36880313.
    Abstract:
    OBJECTIVES: Targeted therapies for blastic plasmacytoid dendritic cell neoplasm (BPDCN) have presented a diagnostic dilemma for differentiating residual BPDCN from reactive plasmacytoid dendritic cells (pDCs) because these conditions have a similar immunoprofile, necessitating discovery of additional diagnostic markers. METHODS: Fifty cases of BPDCN involving bone marrow (26/50) and skin (24/50) as well as other hematologic malignancies (67) and nonneoplastic samples (37) were included. Slides were stained using a double-staining protocol for the following immunohistochemical marker combinations: TCF4/CD123, TCF4/CD56, SOX4/CD123, and IRF8/CD123. RESULTS: The nuclear marker SOX4 is expressed in neoplastic pDCs; in our cohort, SOX4/CD123 showed 100% sensitivity and 98% specificity in distinguishing BPDCN from reactive pDCs and other neoplasms. TCF4/CD56 had a 96% sensitivity and 100% specificity for BPDCN. IRF8 is a nonspecific marker that is positive in BPDCN and pDCs as well as other myeloid malignancies. CONCLUSIONS: The novel immunohistochemical combination SOX4/CD123 distinguishes BPDCN, including CD56-negative BPDCN, from both reactive pDCs and other neoplasms. Because of their high diagnostic sensitivity and specificity, the double-staining marker combinations TCF4/CD123, TCF4/CD56, and SOX4/CD123 can be used to confirm lineage in BPDCN cases and detect minimal/measurable residual disease in tissue specimens.
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