Blastic Plasmacytoid Dendritic Cell Neoplasm: Cytopathologic Findings

Zheng, Gang; Schmieg, John; Guan, Hui; Ali, Syed Z.

doi:10.1159/000332365

Cited by 10 publications

(18 citation statements)

References 26 publications

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“…The neoplastic cells are medium-sized with dispersed chromatin and prominent nucleoli as in cases reported previously [8][9][10], though abundant basophilic cytoplasm and rounded or slightly irregular nucleus are unique on light microscopy. Ultrastructurally, microvillous processes, pseudo-vesicles, a prominent nucleolus, moderate heterochromatin, and parallel arrangements of rER are characteristic of BPDCN, but some of the features can also appear individually in monocytes, plasma cells, and neoplastic cells in chronic lymphocyte leukemia (CLL).…”

Section: Discussionsupporting

confidence: 56%

Morphologic Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Zhang

Dong

et al. 2013

Ultrastructural Pathology

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive lymphoma derived from plasmacytoid dendritic cells or precursor dendritic cells. Despite some 240 reported cases, its morphology and especially ultrastructure has not been satisfactorily studied. A case is reported of a 13 year old boy, who, despite chemotherapy, died within a 12-month period. The electron microscopy findings - microvillous processes, nuclei with slight irregularities, a moderate amount of heterochromatin, and rough endoplasmic reticulum in the form of long, narrow profiles, often in parallel arrangements - taken together, serve to distinguish BPDCN from other neoplastic cells, such as monocytes, plasma cells and the cells of chronic lymphocyte leukemia.

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Section: Discussionsupporting

confidence: 56%

Morphologic Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Zhang

Dong

et al. 2013

Ultrastructural Pathology

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“…13 We found 3 previous reports in the literature regarding the cytomorphologic findings in CSF, touch imprint specimens, and lymph node FNA specimens (Tables 1 and 2). [4][5][6] In 2 of the previous reports, the presence of intracytoplasmic microvacuoles in at least some cells is reported to be a distinctive cytomorphological characteristic of BPDCN. We confirm this cytological finding because we observed intracytoplasmic microvacuoles in 4 cases and there was poor preservation of the cells in the remaining case.…”

Section: Discussionmentioning

confidence: 96%

“…We confirm this cytological finding because we observed intracytoplasmic microvacuoles in 4 cases and there was poor preservation of the cells in the remaining case. 4,6 The differential diagnosis of BPDCN in cytology includes other hematopoietic neoplasms with intermediatesized cells that can exhibit cutaneous involvement and also primary (namely Merkel cell carcinoma) and secondary carcinomas and melanoma (Table 3). In the rare cases without skin involvement, a much broader differential diagnosis must be considered.…”

Section: Discussionmentioning

confidence: 99%

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Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases

Ferreira¹,

Gasparinho

Fonseca³

2015

Cancer Cytopathology

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BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic tumor that was once believed to be derived from natural killer cells and is now recognized as originating from precursors of plasmacytoid dendritic cells. It generally involves the skin and has an aggressive clinical course. Due to its highly malignant behavior, a fast and accurate diagnosis of this condition is of the utmost importance. METHODS: Six cytology specimens from 5 patients diagnosed with BPDCN were reviewed as well as their clinical records. Findings were compared with 3 previously published cases. RESULTS: Two exfoliative cytology specimens (cerebrospinal fluid) and 4 fine-needle aspiration specimens (3 lymph node specimens and 1 cutaneous specimen) were reviewed. The cytomorphological aspects were similar in all cases. The smears were hypercellular with a monotonous population of intermediate-sized cells, dispersed singly or arranged in loose aggregates. The cells had round to oval nuclei, with fine chromatin and prominent nucleoli; the cytoplasm was generally scant, without visible granules. Intracytoplasmic microvacuoles were found in the majority of cases.Four cases were also characterized by flow cytometry, which revealed expression of CD 123, CD56 and/or CD4. Fineneedle aspiration was used in 1 case for primary diagnosis. Histological confirmation was available in all cases. CONCLU-SIONS: BPDCN is a highly malignant neoplasm with a poor outcome. Cytology, in association with flow cytometry immunophenotyping and clinical history, is a reliable method with which to establish the diagnosis. Cancer Cytopathol 2016;124:196-202.

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“…Very importantly, the first cycle must be given in the inpatient hospital setting, in order to adequately monitor for CLS and other toxicities; remaining cycles may be considered for inpatient vs outpatient setting based on provider team discretion. 59 In terms of safety, we reviewed CLS in detail. In addition to the "black box" warning for CLS, the other 2 notable warnings/ precautions are: (1) drug infusion hypersensitivity reaction in 46% and (2) hepatotoxicity in 88%.…”

Section: Fda Approval Package Insert Labeling Indicationmentioning

confidence: 99%

Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm

Pemmaraju¹,

Konopleva

2020

Blood Advances

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and clinically challenging hematologic malignancy with dismal outcomes. With a median age of ∼70 years, the majority of patients with BPDCN have experienced historically suboptimal responses with intensive chemotherapy regimens. The major scientific breakthrough in this field was the recognition of overexpression of a surface receptor, CD123/interleukin 3 (IL-3) receptor α, in all patients. Importantly, a novel therapeutic agent consisting of a truncated diphtheria toxin (DT) payload fused to recombinant human IL-3 was being developed, one that targeted CD123, initially known as DT-IL-3 (later known as SL401; tagraxofusp; tagraxofusp-erzs [Elzonris]). The identification of this agent, and subsequent clinical trials specifically dedicated to patients with BPDCN (including a pilot study, followed by a larger phase 1/2 multicenter study [90% overall response rate [ORR] in frontline and 67% ORR in relapsed/refractory setting]), in part led to approval of tagraxofusp-erzs on 21 December 2018. Tagraxofusp-erzs was the first agent approved for BPDCN (for patients ages 2 years and older), and importantly, established this drug as the first CD123-targeted agent ever approved. The most notable toxicity of tagraxofusp-erzs is occurrence of the capillary leak syndrome, which occurs frequently at all grades, and has also been observed to be life-threatening, appropriately leading to a US Food and Drug Administration “black box” warning in the package insert. The preclinical and clinical aspects of drug development of tagraxofusp-erzs as monotherapy leading to drug approval are reviewed herein, with discussion of future directions of this novel agent, including consideration for rational combinations in BPDCN and beyond.

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Blastic Plasmacytoid Dendritic Cell Neoplasm: Cytopathologic Findings

Cited by 10 publications

References 26 publications

Morphologic Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Morphologic Characteristics of Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report

Cytomorphological features of blastic plasmacytoid dendritic cell neoplasm on FNA and cerebrospinal fluid cytology: A review of 6 cases

Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm

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