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Yavuz Sahin, Y Lynn Wang, Jianming Pei, Nashwa Mansoor, Michael Styler, Joseph R Testa, Reza Nejati
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with an aggressive clinical course and poor prognosis. The genetic abnormalities in BPDCN are heterogeneous; therefore, its molecular pathogenesis and the prognostic importance of genomic alterations associated with the disease are not well defined. Here we report a case of BPDCN with a novel AFF4::IRF1 fusion predicted to lead to a loss-of-function of the IRF1 tumor suppressor, somatic mutations of ASXL1, TET2, and MYD88, as well as multiple intrachromosomal deletions...
December 25, 2023: International Journal of Molecular Sciences
#22
JOURNAL ARTICLE
Evgenii Shumilov, Paolo Mazzeo, Susanne Ghandili, Axel Künstner, Sören Weidemann, Yara Banz, Philipp Ströbel, Matthias Pollak, Lina Kolloch, Helmut Beltraminelli, Andrea Kerkhoff, Jan-Henrik Mikesch, Christoph Schliemann, Detlef Haase, Gerald Wulf, Myriam Legros, Georg Lenz, Laurence Feldmeyer, Thomas Pabst, Hanno Witte, Niklas Gebauer, Ulrike Bacher
Blastic plasmacytoid dendritic cell neoplasm (BPDCN), a rare malignancy derived from plasmacytoid dendritic cells, can mimic both acute leukemia and aggressive T-cell lymphoma. Therapy of this highly aggressive hematological disease should be initiated as soon as possible, especially in light of novel targeted therapies that have become available. However, differential diagnosis of BPDCN remains challenging. This retrospective study aimed to highlight the challenges to timely diagnoses of BPDCN. We documented the diagnostic and clinical features of 43 BPDCN patients diagnosed at five academic hospitals from 2001-2022...
January 9, 2024: Annals of Hematology
#23
JOURNAL ARTICLE
Juanjuan Song, Weiya Li, Yanliang Bai, Pan Zhou, Junwei Niu, Xiaona Niu, Ying Liu, Xiaobo Liu, Emmanuel Kwateng Drokow, Kai Sun, Hu Zhou
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive myeloid malignancy which characteristically expresses an atypical phenotype including CD123+, CD56+, and CD4+. We are aimed to investigate the clinical and prognostic characteristics of AML patients exhibiting BPDCN-like immunophenotype and provide additional insights for risk stratification of AML. A total of 241 newly diagnosed AML patients were enrolled in this retrospective study and categorized into BPDCN-like positive (n = 125)/negative (n = 116) groups, determined by the present with CD123+ along with either CD56+ or CD4+, or both...
January 6, 2024: Annals of Hematology
#24
REVIEW
Gerardo Cazzato, Marialessandra Capuzzolo, Emilio Bellitti, Giovanni De Biasi, Anna Colagrande, Katia Mangialardi, Francesco Gaudio, Giuseppe Ingravallo
Blastic Plasmacytoid Dendritic Cell Neoplasms (BPDCNs) are a rare, highly aggressive hematological malignant neoplasm that primarily involve the skin, bone marrow, lymph nodes and even extra-nodal sites. The rarity and relative poor description of cases in the literature make it necessary to review and further studies that deeply investigate this entity not only in a histopathological but also molecular field. In August-September 2023, we searched MEDLINE, PubMed and Scopus for randomized controlled trials (RCTs), narrative and systematic reviews, meta-analyses, observational studies (either longitudinal or retrospective), and case series published in English in the last 25 years using the keywords BPDCN, PDCs, Blastic NK-cell lymphoma, agranular CD4+ NK leukemia/lymphoma, agranular CD4+ CD56+ hematodermic neoplasm/tumor...
December 8, 2023: Hematology Reports
#25
Ankit Mitesh Shah, Denise Pereira, Julio Poveda, Trent Peng Wang
Venetoclax is an approved treatment for relapsed/refractory Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN). We report a unique case of venetoclax monotherapy used for front-line induction and as a bridge to allogeneic hematopoietic stem cell transplantation (HCT). Venetoclax therapy resulted in rapid complete resolution of skin lesions, however, treatment interruption due to neutropenia led to brisk cancer recurrence. Fortunately, the patient responded to re-challenge and was able to undergo HCT. Venetoclax is active in the first-line treatment setting for BPDCN, however its effect on blood counts and durability of response should be further studied...
2023: Leukemia Research Reports
#26
Bhvaneet Jhajj, Ryan Henrie, Youness El-Khalidy, Habib Moshref Razavi
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive hematologic malignancy which is associated with a distinctive morphologic appearance. However, the morphology is not specific, and diagnostic characterization requires integration of immunophenotypic and genetic testing. We herein report a case of a 35-year-old female patient who presented with worsening cytopenia. A bone marrow aspirate identified medium-sized blastic cells with perinuclear microvacuoles ("pearl neckless"). Occasional blasts demonstrated a "hand mirror" appearance...
2023: Case Reports in Hematology
#27
Ahmad Al-Alwan, Farhan Khalid, Charmee Vyas, Vishakha Sirpal, Husam Bader
Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN) is a rare hematologic malignancy derived from type 2 Dendritic cells (immature Plasmacytoid dendritic cells). It is an aggressive lymphoma and most commonly presents as nonpruritic cutaneous lesions. It can also involve the bone marrow, lymph nodes, or circulating peripheral blasts. Here we present a 61-year-old female with skin bruises all over her body for the last three months associated with fatigue, night sweats, and unintentional weight loss. Her initial diagnosis was Acute Myeloid Leukemia (AML), but later she was diagnosed with BPDCN on tumor biopsy consistent with CD56+ neoplasm...
2023: Journal of Community Hospital Internal Medicine Perspectives
#28
REVIEW
Juan Peng, Shaolong He, Xingcheng Yang, Liang Huang, Jia Wei
Plasmacytoid dendritic cells (pDCs) are a specific dendritic cell type stemming from the myeloid lineage. Clinically and pathologically, neoplasms associated with pDCs are classified as blastic plasmacytoid dendritic cell neoplasm (BPDCN), mature plasmacytoid dendritic myeloid neoplasm (MPDMN) and pDC expansion in myeloid neoplasms (MNs). BPDCN was considered a rare and aggressive neoplasm in the 2016 World Health Organization (WHO) classification. MPDMN, known as mature pDC-derived neoplasm, is closely related to MNs and was first recognized in the latest 2022 WHO classification, proposing a new concept that acute myeloid leukemia cases could show clonally expanded pDCs (pDC-AML)...
October 18, 2023: Critical Reviews in Oncology/hematology
#29
JOURNAL ARTICLE
Ping Cheng, Lan-Lan Wang, Qiu-Xiang Wang, Jun Guan, Ying Zhou, Bin Hu, Yan Feng, Liang Zou, Hui Cheng
OBJECTIVE: To investigate the efficacy and safety of chemotherapy combined with venetoclax followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT) for the treatment of blastic plasmacytoid dendritic cell neoplasm (BPDCN). METHODS: The clinical data of 3 patients with BPDCN undergoing allo-HSCT in Department of Hematology, Wuhan First Hospital from July 2017 to November 2021 were collected and retrospectively analyzed. RESULTS: Among the 3 patients, there were 1 male and 2 females, aged 27-52 years old...
October 2023: Zhongguo Shi Yan Xue Ye Xue za Zhi
#30
REVIEW
Antonella Bruzzese, Enrica Antonia Martino, Caterina Labanca, Francesco Mendicino, Eugenio Lucia, Virginia Olivito, Antonino Neri, Annalisa Imovilli, Fortunato Morabito, Ernesto Vigna, Massimo Gentile
Tagraxofusp (or SL-401) is a recombinant molecule composed of human interleukin-3 that binds CD123 on neoplastic cells fused to a truncated diphtheria toxin (DT). Tagraxofusp's most significant success has come from studies involving patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN), an aggressive disease that is usually refractory to conventional chemotherapy. Tagraxofusp had an acceptable safety profile and high efficacy in early phase I/II studies on patients with BPDCN. Another phase II study confirmed the good response rates, resulting in Food and Drugs Administration and European Medicine Agency approval of tagraxofusp for the treatment of BPDCN...
October 17, 2023: Hematological Oncology
#31
JOURNAL ARTICLE
Sam Sadigh, Daniel J DeAngelo, Jacqueline S Garcia, Robert P Hasserjian, Christopher B Hergott, Andrew A Lane, Scott B Lovitch, Fabienne Lucas, Marlise R Luskin, Elizabeth A Morgan, Geraldine S Pinkus, Olga Pozdnyakova, Scott J Rodig, Vignesh Shanmugam, Harrison K Tsai, Eric S Winer, David Zemmour, Annette S Kim
In this study, we performed a comprehensive molecular analysis of paired skin and peripheral blood/bone marrow (BM) samples from 17 patients with cutaneous myeloid or cutaneous histiocytic-dendritic neoplasms. The cutaneous manifestations included 10 patients with cutaneous acute myeloid leukemia (c-AML), 2 patients with full or partial Langerhans cell differentiation, 2 patients with blastic plasmacytoid dendritic cell neoplasms (BPDCN), 1 patient with both Langerhans cell differentiation and BPDCN, and 2 patients with full or partial indeterminate dendritic cell differentiation...
October 13, 2023: Modern Pathology
#32
JOURNAL ARTICLE
Hemant S Murthy, Mei-Jie Zhang, Karen Chen, Sairah Ahmed, Uday Deotare, Siddhartha Ganguly, Ankit Kansagra, Fotios V Michelis, Taiga Nishihori, Mrinal M Patnaik, Muhammad Bilal Abid, Mahmoud Aljurf, Yasuyuki Arai, Ulrike Bacher, Talha Badar, Sherif M Badawy, Karen Ballen, Minoo Battiwalla, Amer Beitinjaneh, Nelli Bejanyan, Vijaya R Bhatt, Valerie Inez Brown, Rodrigo Martino, Jean-Yves Cahn, Paul Castillo, Jan Cerny, Saurabh Chhabra, Edward A Copelan, Andrew Daly, Bhagirathbhai Dholaria, Miguel Angel Diaz, César O Freytes, Michael R Grunwald, Shahrukh Hashmi, Gerhard C Hildebrandt, Omer Jamy, Jacinth Joseph, Christopher G Kanakry, Nandita Khera, Maxwell M Krem, Yachiyo Kuwatsuka, Hillard M Lazarus, Lazaros J Lekakis, Hongtao Liu, Dipenkumar Modi, Pashna Munshi, Alberto Mussetti, Neil Palmisiano, Sagar S Patel, David A Rizzieri, Sachiko Seo, Mithun Vinod Shah, Akshay Sharma, Melhem M Solh, Scott R Solomon, Matthew L Ulrickson, Celalettin Ustun, Marjolein W M van der Poel, Leo F Verdonck, John L Wagner, Trent P Wang, Baldeep Wirk, Amer M Zeidan, Mark R Litzow, Partow Kebriaei, Christopher S Hourigan, Daniel J Weisdorf, Wael Saber, Mohamed A Kharfan-Dabaja
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with a poor prognosis and considered incurable with standard conventional chemotherapy. Small observational studies have shown that allogeneic hematopoietic cell transplantation (allo-HCT) offers durable remissions in patients with BPDCN. We conducted an analysis of 164 patients with BPDCN from 78 centers who underwent allo-HCT between 2007-2018 using data reported to the Center for International Blood and Marrow Transplant Research (CIBMTR)...
October 4, 2023: Blood Advances
#33
JOURNAL ARTICLE
B R Schrank, G S Manzar, S Y Wu, J R Gunther, P Fang, E J Jabbour, T Y Lim, N G Daver, M D Cykowski, G N Fuller, D Cachia, C Kamiya-Matsuoka, K H Woodman, C D DiNardo, N Jain, N J Short, K Sasaki, B Dabaja, H M Kantarjian, C C Pinnix
PURPOSE/OBJECTIVE(S): Intrathecal (IT) methotrexate (Mtx) and/or cytarabine (AraC) improve CNS disease control in patients (pts) with hematologic malignancies. There are increasing number of case reports of irreversible, primarily dorsal column myelopathy in pts treated with IT chemotherapy. By describing the largest case series of myelopathy following IT chemotherapy, we aim to raise awareness about this devastating albeit rare complication. MATERIALS/METHODS: We retrospectively reviewed 25 pts with leukemia who developed paraplegia following IT chemotherapy between 2/2006 and 9/2021...
October 1, 2023: International Journal of Radiation Oncology, Biology, Physics
#34
Jinzhi Chen, Xi Zhang, Linlin Ma, Yuan Gao, Zhanli Fu, Meng Liu
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an extremely rare hematopoietic malignancy, which originating from precursors of plasmacytoid dendritic cells. Allogeneic hematopoietic stem cell transplantation (HSCT) is normally considered in the treatment of BPDCN patients to acquire sustained remission. Post-transplant lymphoproliferative disorder (PTLD) is a group of conditions involving abnormal lymphoid cells proliferation in the context of extrinsic immunosuppression after solid organ transplantation (SOT) or HSCT...
2023: Frontiers in Medicine
#35
JOURNAL ARTICLE
Yasuhiro Nagate, Aya Nakaya, Ren Kamimura, Yumiko Hirose, Satoshi Nojima, Jiro Fujita, Eiji Kiyohara, Hirohiko Shibayama
An 84-year-old Japanese man was diagnosed with blastic plasmacytoid dendritic cell neoplasm (BPDCN). We administered combination therapy using venetoclax and azacytidine. We observed neutropenia (Grade 4), thrombocytopenia (Grade 2), and stomatitis (Grade 3). After six cycles of treatment, the BPDCN abnormal cells in the bone marrow specimen almost disappeared, and atypical cells were not detected in a skin biopsy. We propose venetoclax combined with azacytidine as a useful treatment approach in elderly patients, although clinicians should be mindful that therapeutic modifications may be essential to minimize and/or avoid adverse events...
2023: Internal Medicine
#36
JOURNAL ARTICLE
Jiasen He, Miriam B Garcia, Jeremy S Connors, Cesar A Nuñez, Andres E Quesada, Amber Gibson, Michael Roth, Branko Cuglievan, Naveen Pemmaraju, David McCall
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy, especially in pediatrics, that can involve the bone marrow, skin, lymph nodes, and central nervous system (CNS). Given its variable clinical presentation, coupled with an immunohistochemistry pattern (CD4, CD56, TCF4, TCL-1, and CD123 positivity) that differs from other myeloid neoplasms, the diagnosis of BPDCN can be missed. Limited data are available to guide the treatment of pediatric BPDCN. Herein, we report a case of a pediatric patient who had BPDCN with central nervous system, orbital, and skin involvement...
November 1, 2023: Journal of Pediatric Hematology/oncology
#37
Christopher J Fay, Christopher Iriarte, Dorsa Moslehi, Anthony R Sheets, Nicole R LeBoeuf
No abstract text is available yet for this article.
September 2023: JAAD Case Reports
#38
Brenna M Aran, Juanita Duran, Darren Whittemore, Alejandro A Gru
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive malignant hematologic neoplasm arising from plasmacytoid dendritic cells. It is a very rare tumor that constitutes less than 0.1% of all hematologic malignancies. Most patients with BPDCN present clinically with cutaneous lesions as the first sign of disease. Immunophenotypic variability with aberrant marker profiles has been reported. We report a case of a transcription factor 4 (TCF-4) + BPDCN, with negative CD56 expression in an 85-year-old woman with multiple skin nodules...
August 23, 2023: Journal of Cutaneous Pathology
#39
Fang Liu, Fei Qi, Jingya Zhang, Yaqi Tan, Xiuying Zhang
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hostile cutaneous malignancy with dismal prognosis and unknown etiology with rarity. Most patients received traditional chemotherapy only has one year of median survival time. This article reports an 81-year-old male patient with BPDCN who presented with skin manifestations and was diagnosed with positive CD4, CD56, and CD123 immunohistochemical results. Systematic examination revealed lung involvement and cytopenia.
2023: Clinical, Cosmetic and Investigational Dermatology
#40
Jiankun Tong, Sergei Aksenov, Beth M Siegel, Lihong Wei, William H Rodgers
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematologic malignancy that arises from plasmacytoid dendritic cells. BPDCN typically presents with skin lesions and may involve peripheral blood, bone marrow, lymph nodes, or extranodal sites. It usually arises de novo, and some BPDCN cases are associated with or develop into myeloid neoplasms. Here, we report a case of a 57-year-old female presenting with cervical lymphadenopathy and skin rashes during the COVID-19 pandemic in 2021 following multiple types of postmastectomy therapy for breast cancer...
2023: Case Reports in Hematology
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