Plasmocytoid Dendritic Cells Leukemia: A Rare Presentation

Abstract

The plasmacytoid dendritic cells leukemia is a rare hematolymphoid neoplasm with a very poor prognosis, where skin involvement is common. It is a malignant proliferation developed at the expense of hematopoietic cells of the white blood cells, proximate to normal plasmacytoid dendritic cells. The phenotype of tumor cells initially described was CD4 + CD56 +, without T marker expression. CD56-forms have also been reported, even fewer without skin involvement. More specific markers are expressed by tumor cells: CD123, BDCA2, BDCA4. We report an unusual presentation of this entity, but without skin lesions. The diagnosis of plasmacytoid dendritic cells leukemia was made on the cytological and immunophenotypic data given. The patient refused treatment and left hospital against medical advice. He returned four weeks later to emergency in an array of septic shock, he died a few hours after admission. Discussion The nomenclature used to describe this entity (Blastic Dendritic Plasmacytoïd Cell Neoplasm (BPDCN) or derived leukemia of