scholarly journals Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report of a Rare Leukemic Variant

2020 ◽  
pp. 1-3
Author(s):  
Ammar Al-Obaidi ◽  
Ammar Al-Obaidi ◽  
Chloe McBride ◽  
Christopher Dakhil ◽  
Khalil Choucair ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Hematological Malignancies ◽  
Hematological Malignancy ◽  
Early Recognition ◽  
Systemic Disease ◽  
Plasmacytoid Dendritic Cell ◽  
Rapid Progression ◽  
Cell Neoplasm ◽  
Aggressive Clinical Course ◽  
Older Males

Blastic plasmacytoid dendritic cell neoplasm, originally described in 1995, is a very rare hematological malignancy with an aggressive clinical course characterized by a rapid progression to systemic disease via hematogenous dissemination. It represents around 0.5% of all hematologic cancers and typically affects older males. Early recognition remains a challenge as it largely overlaps with other hematological malignancies.

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 A very rare case of FLT3-D835 positive blastic plasmacytoid dendritic cell neoplasm

2020 ◽  
Vol 7 (4) ◽  
pp. 57-62
Author(s):  
Vlad Andrei Cianga ◽  
Cătălin Doru Dănăilă ◽  
Ion Antohe
Keyword(s):  
Dendritic Cell ◽  
Rare Case ◽  
Elderly Population ◽  
Hematological Malignancies ◽  
Plasmacytoid Dendritic Cell ◽  
The Elderly ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Cell Neoplasm ◽  
Immuno Histochemistry

Blastic plasmacytoid dendritic cell neoplasms (BPDCNs) are extremely rare and aggressive hematological malignancies that derive from precursors of plasmacytoid dendritic cells (pDC) and frequently involve skin lesions and bone marrow infiltration. They mostly affect the elderly population and the prognosis is poor with the therapeutic choices currently available. Diagnosis is made with the help of tools such as immuno-histochemistry and flow cytometry. Here, we present a particular case of BPDCN with a positive FLT3-D835 mutation and we discuss the possible impact this may have on the evolution of the disease and response to treatment.

scholarly journals Multicenter analysis of outcomes in blastic plasmacytoid dendritic cell neoplasm offers a pretargeted therapy benchmark

Blood ◽  
2019 ◽  
Vol 134 (8) ◽  
pp. 678-687 ◽  
Author(s):  
Justin Taylor ◽  
Michael Haddadin ◽  
Vivek A. Upadhyay ◽  
Erwin Grussie ◽  
Neha Mehta-Shah ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Clinical Characteristics ◽  
Treatment Guidelines ◽  
Hematologic Malignancy ◽  
Systemic Disease ◽  
Plasmacytoid Dendritic Cell ◽  
New Drugs ◽  
Cell Neoplasm ◽  
Poor Outcomes ◽  
Modern Era

Abstract Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an uncommon hematologic malignancy with poor outcomes. Existing data on the clinical behavior of BPDCN are limited because reported outcomes are from small retrospective series, and standardized treatment guidelines are lacking. The interleukin-3 cytotoxin conjugate tagraxofusp was recently tested in phase 1/2 trials that led to US Food and Drug Administration approval, the first ever for BPDCN. However, because there was no matched internal comparator in this or any clinical study to date, results of BPDCN trials testing new drugs are difficult to compare with alternative therapies. We therefore sought to define the clinical characteristics and outcomes of a group of patients with BPDCN treated at 3 US cancer centers in the modern era but before tagraxofusp was available. In 59 studied patients with BPDCN, the median overall survival from diagnosis was 24 months, and outcomes were similar in patients with “skin only” or with systemic disease at presentation. Intensive first-line therapy and “lymphoid-type” chemotherapy regimens were associated with better outcomes. Only 55% of patients received intensive chemotherapy, and 42% of patients underwent stem cell transplantation. Clinical characteristics at diagnosis associated with poorer outcomes included age >60 years, abnormal karyotype, and terminal deoxynucleotidyltransferase (TdT) negativity in the BPDCN cells. We also identified disease responses to pralatrexate and enasidenib in some patients. This study highlights poor outcomes for patients with BPDCN in the modern era and the need for new treatments. Outcomes from ongoing clinical trials for BPDCN can be evaluated relative to this contemporary cohort.

scholarly journals Blastic Plasmacytoid Dendritic Cell Neoplasm: Progress in Cell Origin, Molecular Biology, Diagnostic Criteria and Therapeutic Approaches

2021 ◽  
Author(s):  
Wei Cheng ◽  
Tian-tian Yu ◽  
Ai-ping Tang ◽  
Ken He Young ◽  
Li Yu
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Therapeutic Approaches ◽  
Poor Overall Survival ◽  
Aberrant Expression ◽  
Cell Antigen ◽  
New Therapeutic Approaches ◽  
Cell Neoplasm ◽  
Aggressive Clinical Course ◽  
Blood Dendritic Cell

SummaryBlastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with poor overall survival. BPDCN is derived from plasmacytoid dendritic cells (pDCs) and its pathogenesis is unclear. The tumor cells show aberrant expression of CD4, CD56, interleukin-3 receptor alpha chain (CD123), blood dendritic cell antigen 2 (BDCA 2/CD303), blood dendritic cell antigen 4 (BDCA4) and transcription factor (E protein) E2-2 (TCF4). The best treatment drugs are based on experience by adopting those used for either leukemia or lymphoma. Relapse with drug resistance generally occurs quickly. Stem cell transplantation after the first complete remission is recommended and tagraxofusp is the first targeted therapy. In this review, we summarize the differentiation of BPDCN from its cell origin, its connection with normal pDCs, clinical characteristics, genetic mutations and advances in treatment of BPDCN. This review provides insights into the mechanisms of and new therapeutic approaches for BPDCN.

Tagraxofusp and anti-CD123 in blastic plasmacytoid dendritic cell neoplasm: a new hope

2020 ◽  
Vol 111 (5) ◽  
Author(s):  
Delia Cangini ◽  
Paolo Silimbani ◽  
Alessandro Cafaro ◽  
Maria B. Giannini ◽  
Carla Masini ◽  
...  
Keyword(s):  
Dendritic Cell ◽  
Plasmacytoid Dendritic Cell ◽  
Cell Neoplasm
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