Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: 10‐Color flow cytometry diagnosis and HyperCVAD therapy

Abstract

Few studies describe the comprehensive immunophenotypic pattern of blastic plasmacytoid dendritic cell neoplasm (BPDCN) in the bone marrow and its treatment. This retrospective analysis evaluates the diagnostic flow cytometry (FCM) pattern and outcome of nine patients diagnosed with BPDCN. A four‐tube 10‐color FCM panel used for diagnosis of acute leukemia (AL), showed cells in the blast gate (CD45dim/low SSC) and were positive for CD4(bright), CD33(dim), CD56(heterogenous), CD123(bright), CD36, CD38, HLA‐DR, CD71. Seven patients received front‐line induction therapy with HyperCVAD with an overall response rate of 86%. Five of six responders underwent planned allogeneic hematopoietic cell transplantation (allo‐HCT). For a median follow up of 13.3 months, the 1‐year disease free survival and overall survival were 56 and 67%, respectively. An accurate diagnosis of BPDCN can be made by 10‐color FCM using a four‐tube AL panel demonstrating a characteristic pattern of antigen expression. Front‐line induction chemotherapy with HyperCVAD can yield high remission rates, but allo‐HCT is required for long‐term durable remissions. Am. J. Hematol. 91:283–286, 2016. © 2015 Wiley Periodicals, Inc.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm Extensively Studied by Flow Cytometry and Immunohistochemistry

“…However, unlike the majority of lymphoid malignancies, conventional chemotherapy alone does not appear to be sufficient to ensure durable long-term remissions, with an early relapse rate of about 60% of patients achieving CR [17, 21, 28]. As such, our patient demonstrated an excellent response to HyperCVAD chemotherapy that was further consolidated with allogeneic HSCT.…”

“…A small percentage of BPDCN is conversely characterized by leukemic presentation at diagnosis [16–21]. Within the spectrum of the disease, different maturation stages of BPDCN have been postulated based on the expression of CD34 and CD117.…”

Blastic Plasmacytoid Dendritic Cell Neoplasm: State of the Art and Prospects

“…BPDCN is characterized by an inherent resistance to standard chemotherapies. Treatment responses are mostly transient, the overall outcome being general very poor in general [32,54,92]. Given the rarity of the disease, the available data on BPDCN therapy mainly derive from retrospective studies.…”

“…In general, intensive induction regimens (e.g., hyperCVAD) are considered more effective compared to standard therapies (e.g., CHOP-like) [39,92,93]. In general, ALL-like treatments seem to be more effective in term of response rates than AML-like induction therapies [32,54,92].…”

A case of disseminated blastic plasmocytoid dendritic cell neoplasm

“…A fluorodeoxyglucose (FDG)‐positron emission tomography (PET) scan showed multiple foci of intense metabolic activity on skin and left inguinal lymph nodes. Patient received multi‐agent induction chemotherapy with fractionated cyclophosphamide, vincristine, adriamycin, and dexamethasone (hyper‐CVAD); skin lesions resolved promptly following the first treatment course and bone marrow aspiration documented morphological complete remission with positive cytofluorimetric minimal residual disease (MRD 0.01%). Due to the unavailability of fully matched or haploidentical sibling donors, a donor search in the international bone marrow donor registries was initiated, but was ultimately unsuccessful.…”