Article
Medicine, Research & Experimental
Xinmei Dang, Di Zhou, Lingjun Meng, Lintao Bi
Summary: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive hematodermic malignancy with unclear pathogenesis, lacking standard treatments and having a poor prognosis. Genetic studies in BPDCN patients may help predict prognosis and develop better targeted therapies.
JOURNAL OF INTERNATIONAL MEDICAL RESEARCH
(2021)
Review
Oncology
Hongyan Liao, Jiang Yu, Yu Liu, Sha Zhao, Huanling Zhu, Dongsheng Xu, Nenggang Jiang, Qin Zheng
Summary: This case demonstrates for the first time that prominent pDC proliferation can be associated with lymphoid neoplasms and can exhibit blastic morphology and immunophenotype. The underlying mechanism of the coexistence of these two blastic populations remains unknown.
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
(2022)
Letter
Oncology
Ruth-Miriam Koerber, Stefanie A. E. Held, Maria Vonnahme, Georg Feldmann, Joerg Wenzel, Ines Guetgemann, Peter Brossart, Annkristin Heine
Summary: Blastic plasmacytoid dendritic-cell neoplasm (BPDCN) is an extremely rare disease with origins in dendritic cells, posing challenges in both diagnosis and treatment. It is difficult to distinguish from other leukemic conditions and lacks clear therapeutic standards.
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
(2022)
Article
Oncology
C. Camero Yin, Naveen Pemmaraju, M. James You, Shaoying Li, Jie Xu, Wei Wang, Zhenya Tang, Omar Alswailmi, Kapil N. Bhalla, Muzaffar H. Qazilbash, Marina Konopleva, Joseph D. Khoury
Summary: Mutation and protein-level profiling have expanded our understanding of the pathogenesis of blastic plasmacytoid dendritic cell neoplasm (BPDCN), revealing a high prevalence of somatic mutations involving epigenetic regulators and RNA splicing factors, along with frequent mutations in genes such as ETV6 and IKZF1. Older age, multiple mutations, and mutations in the DNA methylation pathway are poor prognostic factors in BPDCN patients.
Article
Oncology
Peter-Martin Bruch, Sascha Dietrich, Herve Finel, Ariane Boumendil, Hildegard Greinix, Thomas Heinicke, Wolfgang Bethge, Dietrich Beelen, Christoph Schmid, Hans Martin, Luca Castagna, Christof Scheid, Kerstin Schaefer-Eckart, Joerg Bittenbring, Juergen Finke, Henrik Sengeloev, Mael Heiblig, Jan Cornelissen, Patrice Chevallier, Mohamad Mohty, Stephen Robinson, Silvia Montoto, Peter Dreger
Summary: This retrospective study analyzed the outcomes of 162 adult patients with BPDCN who underwent a first HCT. The study found that MAC (especially TBI-based) significantly improved the prognosis of alloHCT recipients, and autoHCT could be considered for patients who are not eligible for MAC.
Article
Pathology
Luisa Lorenzi, Silvia Lonardi, Donatella Vairo, Andrea Bernardelli, Michela Tomaselli, Mattia Bugatti, Sara Licini, Mariachiara Arisi, Lorenzo Cerroni, Alessandra Tucci, William Vermi, Silvia Clara Giliani, Fabio Facchetti
Summary: This study identifies EC as a novel pDC marker of diagnostic relevance in BPDCN. The results suggest a scenario where malignant pDCs promote the blunting of IFN-I signaling through EC-driven signaling, leading to a poorly immunogenic tumor microenvironment.
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2021)
Review
Medicine, General & Internal
Hyo-jae Lee, Hye Mi Park, So Yeon Ki, Yoo-Duk Choi, Sook Jung Yun, Hyo Soon Lim
Summary: This case describes a rare presentation of BPDCN in the breast parenchyma, with no previous reports on the radiologic features of the disease within breast tissue. The patient was diagnosed using diagnostic breast imaging tools and underwent chemotherapy with peripheral blood stem cell transplantation, achieving complete remission.
Review
Oncology
Xavier Roussel, Francine Garnache Ottou, Florian Renosi
Summary: Plasmacytoid dendritic cells (pDC) are the main type I interferon-producing cells in humans and are capable of regulating innate and adaptive immune responses. Tumor infiltration by pDC is well-documented in various cancers and is associated with unfavorable clinical outcomes. While Blastic Plasmacytoid Dendritic Cells Neoplasm (BPDCN) is a well-described aggressive leukemia derived from pDCs, the understanding of tumor infiltration by mature pDCs in Myeloid Neoplasms (MN) is limited. This review aims to provide a comprehensive overview of pDC biology in MN, emphasizing the need for further research into pDC oncogenesis and immune responses for better therapeutic strategies.
Article
Medicine, Research & Experimental
Wei Cheng, Tian-tian Yu, Ai-ping Tang, Ken He Young, Li Yu
Summary: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy derived from plasmacytoid dendritic cells with unclear pathogenesis. Treatment is based on leukemia or lymphoma experience, relapse is quick with drug resistance.
CURRENT MEDICAL SCIENCE
(2021)
Article
Hematology
Corinn Small, Soham Mukerjee, Diwash Jangam, Sumanth Gollapudi, Kunwar Singh, David L. Jaye, Phyu P. Aung, Christiane Querfeld, Keluo Yao, Karen M. Chisholm, Sheeja Pullarkat, Sa Wang, Alejandro Gru, Mohammad Hussaini, Tracy I. George, Robert S. Ohgami
Summary: In this study, the exome sequence data of 9 BPDCN cases were analyzed. Results showed significant genetic changes related to tobacco exposure, aging, nucleotide excision repair deficiency, UV exposure, and endogenous deamination. These findings suggest that environmental and endogenous genetic changes may play a central role in the oncogenesis of BPDCN.
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2023)
Article
Medicine, General & Internal
Li Zhang, Yidong Wang, Mingming Lu, Mengdan Shen, Zhao Duan
Summary: This study presents a case of a 37-year-old pregnant woman with Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). It suggests that pregnant women diagnosed with BPDCN in the third trimester should promptly terminate the pregnancy for further treatment.
Article
Oncology
Wei Wang, Jie Xu, Joseph D. Khoury, Naveen Pemmaraju, Hong Fang, Roberto N. Miranda, C. Cameron Yin, Siba El Hussein, Fuli Jia, Zhenya Tang, Shimin Hu, Marina Konopleva, L. Jeffrey Medeiros, Sa A. Wang
Summary: This study investigated the immunophenotypic and molecular profiles of pDC-AML and BPDCN and found that they have different phenotypes and mutation profiles, indicating that they are two distinct entities.
Article
Biochemistry & Molecular Biology
Giulio Verna, Marina Liso, Elisabetta Cavalcanti, Giusy Bianco, Veronica Di Sarno, Angelo Santino, Pietro Campiglia, Marcello Chieppa
Summary: Dendritic cells can be divided into myeloid dendritic cells and plasmacytoid dendritic cells, which play important roles in regulating immune responses. Inflammatory bowel diseases are characterized by leaky intestinal barrier and translocation of bacterial lipopolysaccharide. It was found that quercetin can modulate dendritic cell responses through a specific molecular pathway.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Oncology
Florian Renosi, Mary Callanan, Christine Lefebvre
Summary: This review discusses the differential diagnosis between Blastic pDC Neoplasm (BPDCN) and Acute Myeloid Leukemia with pDC expansion (pDC-AML) and their genomic features. Understanding the distinct characteristics of these two diseases is crucial for diagnosis and treatment.
Article
Immunology
Tae Jin Yun, Suzu Igarashi, Haoquan Zhao, Oriana A. Perez, Marcus R. Pereira, Emmanuel Zorn, Yufeng Shen, Felicia Goodrum, Adeeb Rahman, Peter A. Sims, Donna L. Farber, Boris Reizis
Summary: Plasmacytoid dendritic cells (pDCs) can recognize live infected cells and produce a unique antiviral immune response, including high amounts of interferons, prolonged and efficient responses to CMV-infected cells, and activation of natural killer cells. Patients with CMV viremia show activated pDCs and increased circulating IFN-I and IFN-III.
SCIENCE IMMUNOLOGY
(2021)