Abstract
<h3>Context</h3> Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare but highly aggressive hematologic malignancy derived from plasmacytoid dendritic cells. Currently, there is no consensus regarding the best therapeutic approach, and the diagnosis of BPDCN remains a challenge. <h3>Objective</h3> We aimed to collect and systematize the currently available evidence about the immunohistochemical (IHC) and flow cytometric (FCM) data in the diagnosis of BPDCN. <h3>Design</h3> A systematic search was carried out in MEDLINE (via PubMed), Embase, Cochrane Central Register of Controlled Trials, Scopus, Web of Science, China National Knowledge Infrastructure, and Latin American, and Caribbean Health Sciences Literature databases for studies published up to 20 April 2021. <h3>Patients or Other Participants</h3> We selected clinical studies reporting on CD4+/CD56+/CD123+ co-expression in BPDCN patients among other markers. <h3>Main Outcomes Measures</h3> Cell-surface marker expression assessed by either IHC or FCM was collected regarding 106 proteins. <h3>Results</h3> The systematic search and data collection are ongoing. Out of 3106 records collected, we identified 115 reported cases from 98 full-text articles. <h3>Conclusions</h3> Based on our preliminary findings, a confident immunophenotypic diagnosis can be made when CD4, CD56, CD123, TCL1, CD43, and CD45 are present and when other lineage-specific antigens are absent (CD19, CD20, CD3, MPO, and CD79a). This work is continuing in collecting all reported data about the diagnostic algorithm of this disorder, which could be used as a template for targeted therapy in BPDCN.
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