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Clinical: 91 yo male with widespread skin nodules, leukocytosis, and cytopenias. Peripheral blood demonstrated medium-sized blastoid cells with grey-blue cytoplasm, occasional cytoplasmic vacuoles, nucleoli, and some nuclear folds. Flow cytometry of peripheral blood: CD4+/CD56+/CD7+/CD33+/dim CD45+ and CD34-. Skin biopsy demonstrated CD4+/ÇD56+ neoplasm. Diagnosis: Blastic plasmacytoid dendritic cell neoplasm with involvement of peripheral blood. This is a clinically aggressive tumor derived from plasmacytoid dendritic cells with high frequency of cutaneous and bone marrow involvement and leukemic dissemination. This has been previously referred to as : Blastic NK-cell lymphoma, agranular CD4+/CD56+ hematodermic neoplasm, agranular CD4+ NK leukemia
Blastic plasmacytoid dendritic cell neoplasm
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Author: Wendy Moreland, MD, John Schmieg, MD, PhD.
Category: Myeloid Neoplasms and acute leukemia (WHO 2016) > Blastic plasmacytoid dendritic cell neoplasm
Published Date: 05/03/2012
Category: Myeloid Neoplasms and acute leukemia (WHO 2016) > Blastic plasmacytoid dendritic cell neoplasm
Published Date: 05/03/2012
Clinical: 91 yo male with widespread skin nodules, leukocytosis, and cytopenias. Peripheral blood demonstrated medium-sized blastoid cells with grey-blue cytoplasm, occasional cytoplasmic vacuoles, nucleoli, and some nuclear folds. Flow cytometry of peripheral blood: CD4+/CD56+/CD7+/CD33+/dim CD45+ and CD34-. Skin biopsy demonstrated CD4+/ÇD56+ neoplasm. Diagnosis: Blastic plasmacytoid dendritic cell neoplasm with involvement of peripheral blood. This is a clinically aggressive tumor derived from plasmacytoid dendritic cells with high frequency of cutaneous and bone marrow involvement and leukemic dissemination. This has been previously referred to as : Blastic NK-cell lymphoma, agranular CD4+/CD56+ hematodermic neoplasm, agranular CD4+ NK leukemia
