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.
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
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)
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
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
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
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
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.
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)
Article
Dermatology
Brenna M. Aran, Juanita Duran, Darren Whittemore, Alejandro A. Gru
Summary: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive hematologic malignancy originating from plasmacytoid dendritic cells. Most patients initially present with cutaneous lesions. We report a case of TCF-4+ BPDCN with negative CD56 expression in an 85-year-old female with multiple skin nodules. BPDCN poses challenges in its diagnosis due to its unusual morphologic variants and phenotypes.
JOURNAL OF CUTANEOUS PATHOLOGY
(2023)
Article
Medical Laboratory Technology
Xiaofang Zhang, Yiping Wu, Chen Li, Keming Shen, Ruimin Li
Summary: The aim of this study was to investigate the clinical characteristics and diagnosis of acute myeloid leukemia with CD56- blastic plasmacytoid dendritic cell neoplasm. Three cases of elderly men with acute myeloid leukemia were analyzed retrospectively, and their bone marrow features suggested the diagnosis of acute myeloid leukemia with blastic plasmacytoid dendritic cell neoplasm. Flow cytometry and second generation sequencing were performed to detect abnormalities in myeloid cells and abnormal plasmacytoid dendritic cells, as well as gene mutations in RUNX1 and DNMT3A.
CLINICAL LABORATORY
(2023)
Article
Medical Laboratory Technology
Vincent H. J. van der Velden, Frank Preijers, Ulrika Johansson, Theresia M. Westers, Alan Dunlop, Anna Porwit, Marie C. Bene, Peter Valent, Jeroen te Marvelde, Orianne Wagner-Ballon, Uta Oelschlaegel, Leonie Saft, Sharham Kordasti, Robin Ireland, Eline Cremers, Canan Alhan, Carolien Duetz, Willemijn Hobo, Nicolas Chapuis, Michaela Fontenay, Peter Bettelheim, Lisa Eidenshink-Brodersen, Patricia Font, Michael R. Loken, Sergio Matarraz, Kiyoyuki Ogata, Alberto Orfao, Katherina Psarra, Dolores Subira, Denise A. Wells, Matteo G. Della Porta, Kate Burbury, Frauke Bellos, Elisabeth Weiss, Wolfgang Kern, Arjan van de Loosdrecht
Summary: This article reviews the progress of flow cytometry (FCM) in the diagnosis of myelodysplastic syndrome (MDS) in recent years, and provides optimal methods and recommendations for sample processing, antibody panels and fluorochromes, and current hardware technologies. These recommendations will facilitate the appropriate application of FCM assays in the diagnostic workup of MDS patients, but further standardization and harmonization are needed.
CYTOMETRY PART B-CLINICAL CYTOMETRY
(2023)
Article
Medical Laboratory Technology
Theresia M. Westers, Leonie Saft, Vincent H. J. van der Velden, Jeroen G. te Marvelde, Alan Dunlop, Robin Ireland, Peter Valent, Anna Porwit, Marie C. Bene, Arjan A. van de Loosdrecht
Summary: This study described the diagnostic workflow of six cases using multimodal integrated diagnostics and demonstrated the application and added value of flow cytometry in patients with myelodysplastic syndrome.
CYTOMETRY PART B-CLINICAL CYTOMETRY
(2023)
Letter
Hematology
Marta Davidson, Florence Wong, Mostafa Atri, Hassan Sibai, Dawn Maze, Verna Cheung, Jeannie Callum, Eshetu G. Atenafu, Vikas Gupta
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Hematology
Naveen Pemmaraju, Hagop Kantarjian, Kendra Sweet, Eunice Wang, Jayastu Senapati, Nathaniel R. Wilson, Marina Konopleva, Arthur E. Frankel, Vikas Gupta, Ruben Mesa, Matthew Ulrickson, Edward Gorak, Sumeet Bhatia, Tulin Budak-Alpdogan, James Mason, Maria Teresa Garcia-Romero, Norma Lopez-Santiago, Gabriela Cesarman-Maus, Pankit Vachhani, Sangmin Lee, Vijaya Raj Bhatt, William Blum, Roland B. Walter, Dale Bixby, Ivana Gojo, Madeleine Duvic, Raajit K. Rampal, Marcos de Lima, James Foran, Amir T. Fathi, Aric Cameron Hall, Meagan A. Jacoby, Jeffrey Lancet, Gabriel Mannis, Anthony S. Stein, Alice Mims, David Rizzieri, Rebecca Olin, Alexander Perl, Gary Schiller, Paul Shami, Richard M. Stone, Stephen Strickland, Matthew J. Wieduwilt, Naval Daver, Farhad Ravandi, Sumithira Vasu, Monica Guzman, Gail J. Roboz, Joseph Khoury, Muzaffar Qazilbash, Phyu P. Aung, Branko Cuglievan, Yazan Madanat, Mohamed A. Kharfan-Dabaja, Anna Pawlowska, Justin Taylor, Martin Tallman, Prajwal Dhakal, Andrew A. Lane
Summary: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with frequent cutaneous involvement and potential invasion of other compartments. CD123 is a targetable surface marker in BPDCN, and efforts are being made to explore novel approaches targeting CD123 and other targets. The North American BPDCN Consortium (NABC) was formed to define the current standard of care and identify research questions and future directions in BPDCN.
Article
Hematology
Ram Vasudevan Nampoothiri, Kenny Tang, Andre Schuh, Wilson Lam, Dawn Maze, Fotios V. Michelis, Steven Chan, Vikas Gupta, Dennis Kim, Rajat Kumar, Jeffrey Howard Lipton, Jonas Mattsson, Mark Minden, Aaron Schimmer, Hassan Sibai, Auro Viswabandya, Karen Yee, Tracy Murphy, Arjun D. Law
Summary: Inconclusive cytogenetic analysis may not be an independent prognostic indicator in patients with acute myeloid leukemia (AML). Molecular abnormalities detected through NGS or whole genome sequencing are more likely to be informative in these patients.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2023)
Article
Hematology
Guru Subramanian Guru Murthy, Soyoung Kim, Noel Estrada-Merly, Muhammad Bilal Abid, Mahmoud Aljurf, Amer Assal, Talha Badar, Sherif M. Badawy, Karen Ballen, Amer Beitinjaneh, Jan Cerny, Saurabh Chhabra, Zachariah DeFilipp, Bhagirathbhai Dholaria, MiguelAngel Diaz Perez, Shatha Farhan, Cesar O. Freytes, Robert Peter Gale, Siddhartha Ganguly, Vikas Gupta, Michael R. Grunwald, Nada Hamad, Gerhard C. Hildebrandt, Yoshihiro Inamoto, Tania Jain, Omer Jamy, Mark Juckett, Matt Kalaycio, Maxwell M. Krem, Hillard M. Lazarus, Mark Litzow, Reinhold Munker, Hemant S. Murthy, Sunita Nathan, Taiga Nishihori, Guillermo Orti, Sagar S. Patel, Marjolein van der Poel, David A. Rizzieri, Bipin N. Savani, Sachiko Seo, Melhem Solh, Leo F. Verdonck, Baldeep Wirk, Jean A. Yared, Ryotaro Nakamura, Betul Oran, Bart Scott, Wael Saber
Summary: Allogeneic hematopoietic cell transplantation (allo-HCT) is the only curative treatment for myelofibrosis. However, the optimal conditioning regimen for this procedure is still unknown.
Article
Oncology
John Mascarenhas, Marina Kremyanskaya, Andrea Patriarca, Francesca Palandri, Timothy Devos, Francesco Passamonti, Raajit K. Rampal, Adam J. Mead, Gabriella Hobbs, Joseph M. Scandura, Moshe Talpaz, Nikki Granacher, Tim C. P. Somervaille, Ronald Hoffman, Marielle J. Wondergem, Mohamed E. Salama, Gozde Colak, Jike Cui, Jean-Jacques Kiladjian, Alessandro M. Vannucchi, Srdan Verstovsek, Natalia Curto-Garcia, Claire Harrison, Vikas Gupta
Summary: In patients with myelofibrosis who are naive to JAKi treatment, the rational combination of the BET inhibitor pelabresib and ruxolitinib showed good tolerability and durable improvements in spleen and symptom burden.
JOURNAL OF CLINICAL ONCOLOGY
(2023)
Article
Oncology
Francesca Palandri, Haifa Kathrin Al-Ali, Paola Guglielmelli, Mike W. Zuurman, Rajendra Sarkar, Vikas Gupta
Summary: Bone marrow fibrosis (BMF) is a negative prognostic factor for myelofibrosis (MF). The JUMP trial investigated the safety and efficacy of the JAK1/JAK2 inhibitor ruxolitinib in symptomatic MF patients. This post hoc analysis found that patients with low-grade fibrosis (LGF) had better response rates and survival estimates compared to patients with high-grade fibrosis (HGF). Early initiation of ruxolitinib therapy was associated with increased response rates in all patients.
Article
Oncology
Nora-Medea Messerich, Narasimha Rao Uda, Thomas Volken, Sergio Cogliatti, Thomas Lehmann, Andreas Holbro, Rudolf Benz, Lukas Graf, Vikas Gupta, Wolfram Jochum, Izadora Demmer, Tata Nageswara Rao, Tobias Silzle
Summary: In myelofibrosis, the C-reactive protein (CRP)/albumin ratio (CAR) and the Glasgow Prognostic Score (GPS) add independent prognostic information. Assessing CRP and albumin helps to identify a vulnerable population of MF patients, which eludes current prognostic models, even if the presence of high-risk mutations is considered. Further evaluation of albumin and CRP as prognostic markers in MF is warranted.
Article
Hematology
Roni Tamari, Donal P. Mclornan, Kwang Woo Ahn, Noel Estrada-Merly, Juan Carlos Hernandez-Boluda, Sergio Giralt, Jeanne Palmer, Robert Peter Gale, Zachariah Defilipp, David I. Marks, Marjolein van der Poel, Leo F. Verdonck, Minoo Battiwalla, Miguel Angel Diaz, Vikas Gupta, Haris Ali, Mark Robert Litzow, Hillard M. Lazarus, Usama Gergis, Asad Bashey, Jane Liesveld, Shahrukh Hashmi, Jeffrey J. Pu, Amer Beitinjaneh, Christopher Bredeson, David Rizzieri, Bipin N. Savani, Muhammad Bilal Abid, Siddhartha Ganguly, Vaibhav Agrawal, Vera Ulrike Bacher, Baldeep Wirk, Tania Jain, Corey Cutler, Mahmoud Aljurf, Tamila Kindwall-Keller, Mohamed A. Kharfan-Dabaja, Gerhard C. Hildebrandt, Attaphol Pawarode, Melhem M. Solh, Jean A. Yared, Michael R. Grunwald, Sunita Nathan, Taiga Nishihori, Sachiko Seo, Bart L. Scott, Ryotaro Nakamura, Betul Oran, Tomasz Czerw, Ibrahim Yakoub-Agha, Wael Saber
Summary: A prognostic model was developed for myelofibrosis patients undergoing allogeneic hematopoietic cell transplantation. Factors such as patient age, donor compatibility, and hemoglobin levels were found to be associated with mortality risk. The proposed scoring system could accurately predict overall survival and transplant-related mortality in two large cohorts.
Article
Hematology
Aniket Bankar, Wing C. Chan, Ning Liu, Matthew Cheung, Shabbir Alibhai, Vikas Gupta
Summary: This population-based study found a high prevalence of frailty in patients with myeloproliferative neoplasms (MPN), including essential thrombocythemia, polycythemia vera, and myelofibrosis, even in patients with younger age or minimal comorbidities. Frailty was independently associated with worse overall survival, regardless of age or comorbidities.
Article
Health Care Sciences & Services
Michael Bonares, Lisa W. Le, Camilla Zimmermann, Kristen Wentlandt
Summary: Although patients with nonmalignant diseases have similar palliative care needs to cancer patients, they are less likely to receive specialist palliative care. This study compared referral practices to specialist palliative care among cardiologists, respirologists, and oncologists, and found that the perceived availability of specialist palliative care services was higher for cancer patients compared to non-cancer patients.
JOURNAL OF PAIN AND SYMPTOM MANAGEMENT
(2023)
Article
Oncology
Anne Tierens, Elizabeth Kagotho, Satoru Shinriki, Andrew Seto, Adam C. Smith, Melanie Care, Dawn Maze, Hassan Sibai, Karen W. Yee, Andre C. Schuh, Dennis Dong Hwan Kim, Vikas Gupta, Mark D. Minden, Hirotaka Matsui, Jose-Mario Capo-Chichi
Summary: Inherited DDX41 mutations cause familial predisposition to hematologic malignancies including acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS). However, the genotype-phenotype correlation in DDX41-MDS/AML remains poorly understood.
FRONTIERS IN ONCOLOGY
(2023)
Article
Hematology
Vikas Gupta, John Mascarenhas, Marina Kremyanskaya, Raajit K. Rampal, Moshe Talpaz, Jean-Jacques Kiladjian, Alessandro M. Vannucchi, Srdan Verstovsek, Gozde Colak, Debarshi Dey, Claire Harrison
Summary: A study suggests that pelabresib in combination with ruxolitinib may have higher efficacy in treating JAKi treatment-naive MF patients compared to JAKi monotherapy, with significant improvements in spleen volume reduction and total symptom scores.
Article
Hematology
Anna Nikkarinen, Lavanya Lokhande, Rose-Marie Amini, Mats Jerkeman, Anna Porwit, Daniel Molin, Gunilla Enblad, Arne Kolstad, Riikka Raety, Martin Hutchings, Caroline E. Weibull, Peter Hollander, Sara Ek, Ingrid Glimelius
Summary: The prognostic value of soluble CD163 (sCD163) was investigated in patients with mantle cell lymphoma (MCL). High levels of sCD163 were associated with shorter progression-free survival and shorter overall survival, while low levels of sCD163 indicated a very good prognosis. The presence of M2 macrophages, identified by CD163 expression, was correlated with poor outcomes in MCL patients.
