Blastic plasmacytoid dendritic cell neoplasm in the background of myeloproliferative disorder and chronic lymphocytic leukaemia

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare haematological malignancy defined by concurrent expression of CD4, CD56, BCL-2 and CD123. The disease has a very poor prognosis and there are no well-established treatment guidelines. We describe a case of BPDCN in a 65-year-old female patient with myeloproliferative disorder (essential thrombocythemia) and chronic lymphocytic leukaemia. She presented with rapidly progressive facial and scalp lesions. Skin biopsy confirmed BPDCN and the imaging revealed widespread disease. Patient was started on hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone) and intrathecal methotrexate. Due to progression on initial treatment, she was treated with decitabine and venetoclax (BCL-2 inhibitor). However, patient continued to deteriorate and died after 4 months from initial diagnosis. We emphasise on the clinical features, emerging treatment modalities and prognosis of BPDCN.

Frontline Hyper-CVAD Plus Venetoclax for Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm

“…Similar cases of BPDCN with life-threatening TLS shortly after treatment with chemotherapy or tagraxofusp have been reported 15,16 . DIC has been reported in BPDCN patients, but mostly in elderly adults 17,18 . These findings emphasize the importance of monitoring BPDCN patients for TLS and DIC.…”

“…15,16 DIC has been reported in BPDCN patients, but mostly in elderly adults. 17,18 These findings emphasize the importance of monitoring BPDCN patients for TLS and DIC.…”

Flow Cytometry Profiling of Plasmacytoid Dendritic Cell Neoplasms

“…A pDC population in this setting is more likely to be detected by flow cytometry analysis, which would trigger further evaluation to determine the presence of a concurrent BPDCN along The value of flow cytometry analysis, particularly in the setting of BPDCN minimally involving bone marrow or concurrently presenting with other myeloid neoplasms, has been previously highlighted [11]. In this scenario, the bone marrow involved by the myeloid neoplasm is typically hypercellular, and the BPDCN component may be challenging to identify based on the morphologic assessment of the core biopsy alone [12,19]. A pDC population in this setting is more likely to be detected by flow cytometry analysis, which would trigger further evaluation to determine the presence of a concurrent BPDCN along with the established hematologic neoplasm [12].…”