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

2017 ◽  
Vol 2017 ◽  
pp. 1-6
Author(s):  
Martina Pennisi ◽  
Clara Cesana ◽  
Micol Giulia Cittone ◽  
Laura Bandiera ◽  
Barbara Scarpati ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Lymphoblastic Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Hematopoietic Stem ◽  
Cell Neoplasm ◽  
Specific Antigens ◽  
Aggressive Clinical Course

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+CD56+,CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation.

scholarly journals CD123 as a Biomarker in Hematolymphoid Malignancies: Principles of Detection and Targeted Therapies

Cancers ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3087
Author(s):  
Hanadi El Achi ◽  
Edouard Dupont ◽  
Shilpa Paul ◽  
Joseph D. Khoury
Keyword(s):  
Stem Cells ◽  
T Cells ◽  
Dendritic Cell ◽  
Targeted Therapies ◽  
Systemic Mastocytosis ◽  
Lymphoblastic Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Hematopoietic Stem ◽  
Promising Therapeutic Approach ◽  
Cell Neoplasm

CD123, the α chain of the interleukin 3 receptor, is a cytokine receptor that is overexpressed in multiple hematolymphoid neoplasms, including acute myeloid leukemia, blastic plasmacytoid dendritic cell neoplasm, acute lymphoblastic leukemia, hairy cell leukemia, and systemic mastocytosis. Importantly, CD123 expression is upregulated in leukemic stem cells relative to non-neoplastic hematopoietic stem cells, which makes it a useful diagnostic and therapeutic biomarker in hematologic malignancies. Varying levels of evidence have shown that CD123-targeted therapy represents a promising therapeutic approach in several cancers. Tagraxofusp, an anti-CD123 antibody conjugated to a diphtheria toxin, has been approved for use in patients with blastic plasmacytoid dendritic cell neoplasm. Multiple clinical trials are investigating the use of various CD123-targeting agents, including chimeric antigen receptor-modified T cells (expressing CD123, monoclonal antibodies, combined CD3-CD123 dual-affinity retargeting antibody therapy, recombinant fusion proteins, and CD123-engager T cells. In this review, we provide an overview of laboratory techniques used to evaluate and monitor CD123 expression, describe the strengths and limitations of detecting this biomarker in guiding therapy decisions, and provide an overview of the pharmacologic principles and strategies used in CD123-targeted therapies.

scholarly journals Blastic plasmacytoid dendritic cell neoplasm

2019 ◽  
Vol 18 (4) ◽  
pp. 79-89
Author(s):  
T. T. Valiev ◽  
G. Z. Seregin ◽  
I. N. Serebryakova ◽  
O. A. Chernyshova ◽  
N. A. Kupryshina ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Lymphoblastic Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Molecular Genetic ◽  
Allogeneic Bone Marrow Transplantation ◽  
Clinical Analysis ◽  
Allogeneic Bone ◽  
Molecular Features ◽  
Cell Neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare hematologic malignancy. Our view of the cellular origins of this kind of tumor has been changing dramatically with the emergence of new data on the molecular biological and immunological characteristics of the tumor. This article discusses the clinical features of BPDCN, as well as the cytological, morphological-immunological and molecular genetic criteria for BPDCN diagnosis. Taking into account the rare incidence of BPDCN, as well as its rather complex diagnostic procedure, which requires an extended diagnostic antibody panel, standard methods of therapy have not been developed. Chemotherapy protocols for acute lymphoblastic leukemia and acute myeloid leukemia are used, with/without subsequent autologous/allogeneic bone marrow transplantation, but the results remain unsatisfactory. For the first time in Russian cancer research, this article provides a description of BPDCN in a 14-year-old child. A detailed clinical analysis of this rare tumor is provided, as well as dermatoscopy results and a description of the histological, immunological and molecular features of BPDCN, from the point of view of differential diagnosis. Parents patients agreed to use personal data in research and publications.

scholarly journals Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm

2020 ◽  
Vol 4 (16) ◽  
pp. 4020-4027 ◽  
Author(s):  
Naveen Pemmaraju ◽  
Marina Konopleva
Keyword(s):  
Dendritic Cell ◽  
Hematologic Malignancy ◽  
Phase 1 ◽  
Plasmacytoid Dendritic Cell ◽  
Package Insert ◽  
Drug Approval ◽  
Clinical Aspects ◽  
Patients Âgés ◽  
Cell Neoplasm ◽  
Surface Receptor

Abstract 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.

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

2016 ◽  
Vol 91 (3) ◽  
pp. 283-286 ◽  
Author(s):  
Uday Deotare ◽  
Karen W.L. Yee ◽  
Lisa W. Le ◽  
Anna Porwit ◽  
Anne Tierens ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Color Flow ◽  
Cell Neoplasm

scholarly journals Pathology Quiz Case: Plasmacytoid Dendritic Cell Neoplasm

2014 ◽  
Vol 5 (1) ◽  
pp. ar.2014.5.0085 ◽  
Author(s):  
Quinn A. Dunlap ◽  
Kristine E. Day ◽  
Samuel G. Borak ◽  
Bradford A. Woodworth
Keyword(s):  
Dendritic Cell ◽  
Early Diagnosis ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Current Case ◽  
Systemic Involvement ◽  
Cell Neoplasm ◽  
Putative Origin ◽  
Diagnostic Modalities ◽  
Paranasal Sinus Tumor

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that possesses a heterogenous clinical and immunophenotypic presentation. The current case report describes an interesting and unique presentation of BPDCN as a primary paranasal sinus tumor without evidence of cutaneous or systemic involvement. As such, the report further contributes to the ongoing debate regarding the true putative origin of the neoplasm, as well as highlights the optimal diagnostic modalities, paramount importance of early diagnosis, and vast heterogeneity exhibited by this fascinating malignancy. The atypical presentation described here indicates the manifestations of BPDCN are more heterogenous than previously documented and thus can not be definitively ruled out in the absence of bone marrow, peripheral blood, or cutaneous involvement. Furthermore, atypical neoplastic presentations mandate flow cytometry and adjunctive immunohistochemistry for the definitive diagnosis of BPDCN, and early diagnosis of such neoplasms are critical for rapid initiation of treatment and improved outcomes.

scholarly journals Blastic Plasmacytoid Dendritic Cell Neoplasm: A Rare Case Report with Literature Review

2021 ◽  
Author(s):  
Oza Nikita ◽  
Krishnakumar Rathnam ◽  
Gujral Sumeet ◽  
Susan R. Honey ◽  
S. V. Saju ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Lymphoblastic Leukemia ◽  
Plasmacytoid Dendritic Cell ◽  
Excision Biopsy ◽  
Complete Disappearance ◽  
Peripheral Smear ◽  
Normal Limits ◽  
Hla Dr ◽  
Cell Neoplasm ◽  
Rare Case Report

AbstractBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm for which there are no effective therapies. We present a 70-year-old male patient with multiple reddish painless, nonpruritic, and nonpedunculated nodules over the trunk, forearm, and thighs for a duration of 3 months. The nodules measured 0.5 to 2 cm in diameter. The peripheral smear findings were within normal limits. Excision biopsy was performed. Histomorphology and immunohistochemistry (CD123, CD 56, CD4, HLA-DR, CD43, and CD68) confirmed the diagnosis of BPDCN. Findings of marrow aspiration, biopsy and imaging studies were within normal limits. Patient demonstrated a good response with complete disappearance of all nodules by initial 2 weeks of therapy with a modified Berlin–Frankfurt–Munster (BFM) acute lymphoblastic leukemia (ALL) protocol and has completed 8 doses (LSAP [lincosamides, streptogramins A and pleuromutilins chemotherapy], 5,000 units/m2). The patient tolerated protocol extremely well.

scholarly journals Blastic plasmacytoid dendritic cell neoplasm with skin and bone marrow involvement: Report of three cases

2021 ◽  
Vol 9 (33) ◽  
pp. 10293-10299
Author(s):  
Jiang-Hong Guo ◽  
Hong-Wei Zhang ◽  
Li Wang ◽  
Wei Bai ◽  
Jin-Fen Wang
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Bone Marrow Involvement ◽  
Cell Neoplasm

scholarly journals Features of non-activation dendritic state and immune deficiency in blastic plasmacytoid dendritic cell neoplasm (BPDCN)

2019 ◽  
Vol 9 (12) ◽  
Author(s):  
Hannah C. Beird ◽  
Maliha Khan ◽  
Feng Wang ◽  
Mansour Alfayez ◽  
Tianyu Cai ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dendritic Cell ◽  
Peripheral Blood ◽  
Profile Analysis ◽  
Hematologic Malignancy ◽  
Plasmacytoid Dendritic Cell ◽  
Serum Samples ◽  
Molecular Features ◽  
Cell Neoplasm ◽  
Poor Outcomes

AbstractBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, male-predominant hematologic malignancy with poor outcomes and with just one recently approved agent (tagraxofusp). It is characterized by the abnormal proliferation of precursor plasmacytoid dendritic cells (pDCs) with morphologic and molecular similarities to acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS)/chronic myelomonocytic leukemia (CMML) in its presentation within the bone marrow and peripheral blood. To identify disease-specific molecular features of BPDCN, we profiled the bone marrow, peripheral blood, and serum samples from primary patient samples using an in-house hematologic malignancy panel (“T300” panel), transcriptome microarray, and serum multiplex immunoassays. TET2 mutations (5/8, 63%) were the most prevalent in our cohort. Using the transcriptome microarray, genes specific to pDCs (LAMP5, CCDC50) were more highly expressed in BPDCN than in AML specimens. Finally, the serum cytokine profile analysis showed significantly elevated levels of eosinophil chemoattractants eotaxin and RANTES in BPDCN as compared with AML. Along with the high levels of PTPRS and dendritic nature of the tumor cells, these findings suggest a possible pre-inflammatory context of this disease, in which BPDCN features nonactivated pDCs.

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