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
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
Allison Morin, Flora Kechedjian, Paige Walton, Aran Tavakoli
Summary: BPDCN, a rare hematologic malignancy, has been underrecognized due to renaming and reclassification. Tagraxofusp, the first FDA-approved therapy for BPDCN, can cause serious adverse events like Capillary Leak Syndrome. Nurses should monitor patients for potential hypersensitivity reactions and hepatotoxicity during treatment.
CLINICAL JOURNAL OF ONCOLOGY NURSING
(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)
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.
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
Oncology
Yazan Samhouri, Sorana Ursu, Nina Dutton, Verma Tanvi, Salman Fazal
Summary: BPDCN, a rare and aggressive hematologic malignancy originating from plasmacytoid dendritic cells, lacks consensus treatment recommendations, particularly in relapsed cases. Tagraxofusp, a recently approved therapy, was used in an elderly patient with BPDCN, followed by a combination of azacitidine and venetoclax upon relapse, with good tolerance and positive response. Further real-world experience is needed for Tagraxofusp, while the azacitidine/venetoclax combination shows promise as a potential treatment option for this rare disease.
JOURNAL OF ONCOLOGY PHARMACY PRACTICE
(2021)
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
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
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
Oncology
Naveen Pemmaraju, Kendra L. Sweet, Anthony S. Stein, Eunice S. Wang, David A. Rizzieri, Sumithira Vasu, Todd L. Rosenblat, Christopher L. Brooks, Nassir Habboubi, Tariq Mughal, Hagop Kantarjian, Marina Konopleva, Andrew A. Lane
Summary: In this article, the long-term results of a clinical trial for BPDCN, including treatment-naive and relapsed/refractory patients, were reported. The study showed that TAG monotherapy resulted in high and durable responses in first-line patients with BPDCN, allowing many patients to bridge to stem-cell transplant.
JOURNAL OF CLINICAL ONCOLOGY
(2022)
Review
Medicine, General & Internal
Yemin Wang, Li Xiao, Lili Yin, Lv Zhou, Yanjuan Deng, Huan Deng
Summary: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic disease involving the skin and bone marrow. The immunophenotype of BPDCN is characterized by the expression of CD4, CD56, CD123, TCL-1, and CD303. Current treatment for BPDCN is based on high-dose chemotherapy combined with stem cell transplantation, but targeted therapies have shown great promise. This article focuses on the latest advances in genetics and targeted therapies for BPDCN, providing new ideas for its clinical treatment.
Article
Dermatology
Ying Zhang, Jingshu Xiong, Siqi Li, Yan Li, Xuebao Shao, Wei Zhang, Xiulian Xu, Yiqun Jiang, Jianfang Sun, Hao Chen
Summary: This retrospective study aims to characterize the clinical, histopathological, and immunophenotypic features of BPDCN with cutaneous involvement. The results showed that the morphological and phenotypic features of cutaneous BPDCN are heterogeneous. Notably, E2-2 may serve as a useful marker to definitively diagnose BPDCN.
EUROPEAN JOURNAL OF DERMATOLOGY
(2022)
Article
Pathology
Xin Zhang, Jing Han, Na Zhu, Yuan Ji, Yingyong Hou
Summary: This case report describes a rare case of systemic mastocytosis with aggressive involvement of the gastrointestinal tract. The patient exhibited symptoms of long-term abdominal discomfort and diarrhea, and was successfully treated with avapritinib, resulting in significant clinical improvement.
DIAGNOSTIC PATHOLOGY
(2023)
Review
Oncology
Suvendu Purkait, Sanjeev Gupta, Sameer Bakhshi, Saumyaranjan Mallick
Summary: This article describes the clinicopathological features of three cases of BPDCN, including two with classical immunophenotype and one with an uncommon immunophenotype.
JOURNAL OF CANCER RESEARCH AND THERAPEUTICS
(2022)
