Clinicopathologic retrospective analysis of blastic plasmacytoid dendritic cell neoplasms

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is one of the aggressive rare hematopoietic malignancies with predilection to the skin, primarily found in adults. The precise incidence of BPDCN is difficult to estimate due to constantly changing nomenclature and lack of precise defining criteria prior to the 2008 WHO classification system. There are not many cases described in the literature, what makes the diagnostic process challenging. Skin lesions such as erythematous infiltrates and nodules are usually the first manifestation of the disease. Therefore, in doubtful diagnostic cases, dermatologists should perform histopathological and immunohistochemistry examinations along with hematological and oncological cooperation, as early diagnosis and appropriate treatment is essential for improvement of the disease course. This analysis, despite the small number of patients may provide useful information on the clinical and histopathological features of this rare malignancy.

Spontaneous Regression of Blastic Plasmacytoid Dendritic Cell Neoplasm Following Sepsis by <i>Serratia marcescens</i>: A Case Report and Literature Review

“…To date, four cases of spontaneous remission of BPDCN have been reported (16)(17)(18)(19) in the English literature, and we will review the five total cases, including our present one (Table). Four of the patients were men, and one was a woman.…”

“…SR in BPDCN is rare, partly due to the short history of the disease. To our knowledge, there have been only four reported cases of spontaneous remission of BPDCN (16)(17)(18)(19) in the English literature, none of which were related to sepsis. We herein report the first case of spontaneous regression of BPDCN, with a decrease in tumor cells in the bone marrow following severe sepsis induced by Serratia marcescens.…”

Dendritic Cells and Their Roles in Anti-Tumour Immunity

“…Whether AS DCs and pDC are 2 distinct cell types or differentiation stages of one another is yet to be defined. A rare and highly aggressive acute leukaemia known as Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) involves the malignancy of pDC precursors [48], driven, at least in part by the juxtaposition of the pDC-specific RUNX2 enhancer and the MYC promotor due to the chromosomal translocation (6;8) (p21;q24) [49]. The BPDCN can be reliably identified by immunohistochemical staining with TCF4 and CD123 antibodies [50].…”

A misdiagnosed case of blastic plasmacytoid dendritic cell neoplasm experiencing multiple recurrences who underwent allogeneic stem cell transplantation: a case report

“…Since BPDCN demonstrates some overlapping features with other hematologic malignancies, early tumor detection remains challenging. Therefore, a complete history and a physical examination should be accompanied by imaging modalities, flow cytometry, and immunohistochemistry (IHC) of affected tissues to confirm the diagnosis [ 4 – 6 ].…”