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.
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
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.
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
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
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)
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
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)
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
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)
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
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)
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
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
Pathology
Charlotte Syrykh, Loic Ysebaert, Sarah Pericart, Solene M. Evrard, Fabienne Meggetto, Salim Kanoun, Pierre Brousset, Camille Laurent
Summary: This report describes an unusual case of ALK-positive histiocytosis with an Erdheim-Chester disease-like presentation in a 37-year-old woman with chronic lymphocytic leukaemia. The patient experienced a relapse of CLL 6 months after frontline therapy, but after 4 years of ibrutinib treatment, remains free of both diseases. The study suggests the potential efficacy of BTK inhibitors in histiocytic neoplasms.
Letter
Oncology
Franck Morschhauser, Martin J. S. Dyer, Harriet S. Walter, Alexey V. Danilov, Loic Ysebaert, Daniel James Hodson, Christopher Fegan, Simon A. Rule, John Radford, Guillaume Cartron, Krimo Bouabdallah, Andrew John Davies, Stephen Spurgeon, Nishanthan Rajakumaraswamy, Biao Li, Rita Humeniuk, Xi Huang, Pankaj Bhargava, Juliane M. Jurgensmeier, Gilles Salles
Article
Hematology
Anne-Sophie Michallet, Remi Letestu, Magali Le Garff-Tavernier, Carmen Aanei, Michel Ticchioni, Marie-Sarah Dilhuydy, Fabien Subtil, Valerie Rouille, Beatrice Mahe, Kamel Laribi, Bruno Villemagne, Gilles Salles, Olivier Tournilhac, Alain Delmer, Christelle Portois, Brigitte Pegourie, Veronique Leblond, Cecile Tomowiak, Sophie De Guibert, Frederique Orsini Piocelle, Anne Banos, Philippe Carassou, Guillaume Cartron, Luc-Matthieu Fornecker, Loic Ysebaert, Caroline Dartigeas, Malgorzata Truchan-Graczyk, Jean-Pierre Vilque, Therese Aurran, Florence Cymbalista, Stephane Lepretre, Vincent Levy, Florence Nguyen-Khac, Pierre Feugier
Summary: Research shows that a fixed-duration approach in previously untreated, medically fit patients with CLL can achieve high survival rates and persistent treatment benefit, with low long-term toxicity.
Article
Hematology
Charles Herbaux, Christoph Kornauth, Stephanie Poulain, Stephen J. F. Chong, Mary C. Collins, Rebecca Valentin, Liam Hackett, Olivier Tournilhac, Francois Lemonnier, Jehan Dupuis, Adrien Daniel, Cecile Tomowiak, Kamel Laribi, Loic Renaud, Damien Roos-Weil, Cedric Rossi, Eric Van den Neste, Cecile Leyronnas, Fatiha Merabet, Jean Valere Malfuson, Mourad Tiab, Loic Ysebaert, Samuel Ng, Franck Morschhauser, Philipp B. Staber, Matthew S. Davids
Summary: Novel agent classes like JAK/STAT and T-cell receptor pathway inhibitors, as well as HDAC inhibitors, have shown preclinical activity in T-PLL. BH3 profiling revealed that primary T-PLL cells mainly depend on BCL-2 and MCL-1 proteins for survival. The combination therapy of venetoclax and ruxolitinib demonstrated significant efficacy in refractory T-PLL patients, especially in those with JAK3 mutations.
Article
Public, Environmental & Occupational Health
Herve Ghesquieres, Cedric Rossi, Fanny Cherblanc, Sandra Le Guyader-Peyrou, Fontanet Bijou, Pierre Sujobert, Pascale Fabbro-Peray, Adeline Bernier, Aurelien Belot, Loic Chartier, Luc-Matthieu Fornecker, Isabelle Baldi, Krimo Bouabdallah, Camille Laurent, Lucie Oberic, Nadine Morineau, Steven Le Gouill, Franck Morschhauser, Corinne Haioun, Gandhi Damaj, Stephanie Guidez, Gaelle Laboure, Olivier Fitoussi, Laure Lebras, Remy Gressin, Gilles Salles, Loic Ysebaert, Alain Monnereau
Summary: The lymphoma incidence rates in France have been increasing since the early 80s, but the growth has slowed since 2010 and varies across subtypes. The REALYSA study is a real-life multicentric cohort aimed at studying prognostic factors in lymphoma patients.
Article
Pharmacology & Pharmacy
Fanny Gallais, Loic Ysebaert, Fabien Despas, Sandra De Barros, Lucie Oberic, Ben Allal, Etienne Chatelut, Melanie White-Koning
Summary: This study developed the first PK-PD model for ALC in patients with CLL under ibrutinib treatment. The results of the model suggested that estimated lymphocyte counts in tissues and blood could be used as an early predictor of treatment response in CLL patients.
CLINICAL PHARMACOLOGY & THERAPEUTICS
(2021)
Review
Immunology
Loic Ysebaert, Anne Quillet-Mary, Marie Tosolini, Frederic Pont, Camille Laurent, Jean-Jacques Fournie
Summary: High-definition transcriptomic studies using single-cell RNA sequencing have provided insights into cellular heterogeneity and functionality in solid tumors, offering valuable information for clinical response to immune checkpoint inhibitors. This technology also has the potential to uncover the intricate heterogeneity of ecosystems in different lymphoma entities.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Chemistry, Medicinal
Felicien Le Louedec, Fanny Gallais, Fabienne Thomas, Melanie White-Koning, Ben Allal, Caroline Protin, Loic Ysebaert, Etienne Chatelut, Florent Puisset
Summary: The study found that a limited sampling strategy using the T0-1-2-4 design can accurately estimate ibrutinib exposure, with good accuracy even when removing the 1-hour or 2-hour sampling points. The correlation between actual AUC(IBRU) and C-min, C-ss was poor, and joint analysis of dihydrodiol-ibrutinib metabolite concentrations did not improve predictive performance. At least three samples within the pre-dose and 4-hour post-dose period are necessary for accurate estimation of ibrutinib exposure.
Article
Oncology
Fabien Gava, Carla Faria, Pauline Gravelle, Juan G. Valero, Celia Dobano-Lopez, Renaud Morin, Marine Norlund, Aurelie Gomes, Jean-Michel Lagarde, Cedric Rossi, Julie Bordenave, Laetitia Pieruccioni, Jacques Rouquette, Alba Matas-Cespedes, Jean-Jacques Fournie, Loic Ysebaert, Camille Laurent, Patricia Perez-Galan, Christine Bezombes
Summary: Follicular lymphoma is an incurable type of B cell lymphoproliferative disorder, accounting for a significant percentage of NHL cases. The development of a realistic 3D model offers a promising approach for testing new therapeutic targets.
Letter
Hematology
Gregory Lazarian, Michael Munger, Anne Quinquenel, Marie-Sarah Dilhuydy, Lauren Veronese, Damien Luque Paz, Romain Guieze, Albane Ledoux-Pilon, Jerome Paillassa, Fatiha Merabet, Jean-Philippe Vial, Audrey Bidet, Agathe Waultier Rascalou, Julien Broseus, Damien Roos-Weil, Anne Lavaud, Lysiane Molina, Kamel Laribi, Benedicte Hivert, Chloe Friedrich, Benjamin Carpentier, Loic Ysebaert, Eric Van Den Neste, Lise Willems, Anne Corby, Stephanie Poulain, Virginie Eclache, Eve Maubec, Antoine Martin, Pierre Feugier, Alain Delmer, Fanny Baran-Marszak, Stephane Lepretre, Florence Cymbalista
AMERICAN JOURNAL OF HEMATOLOGY
(2021)
Letter
Oncology
Leopoldine Lapierre, Sarah Pericart, Caroline Protin, Cecile Borel, Loic Ysebaert, Camille Laurent, Lucie Oberic
LEUKEMIA & LYMPHOMA
(2021)
Article
Oncology
Virginie Nerich, Christophe Guyeux, Michel Henry-Amar, Raphael Couturier, Catherine Thieblemont, Vincent Ribrag, Herve Tilly, Corinne Haioun, Rene-Olivier Casasnovas, Franck Morschhauser, Pierre Feugier, David Sibon, Loic Ysebaert, Emmanuelle Nicolas-Virelizier, Florence Broussais-Guillaumot, Gandhi L. Damaj, Jean-Philippe Jais, Gilles Salles, Macha Woronoff-Lemsi, Nicolas Mounier
Summary: This study focused on the health care resource use and associated costs in 1671 long-term NHL survivors in France. The results showed that most survivors utilized health care resources, with hospitalizations and outpatient treatments being major cost drivers. Several factors were found to have significant relationships with health care costs, but rituximab or autologous stem cell transplantation did not affect costs.
Letter
Dermatology
V. Sibaud, A. Brun, N. Meyer, L. Oberic, L. Lamant, L. Ysebaert
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2022)
Meeting Abstract
Pharmacology & Pharmacy
T. Pacheco-Paez, C. Conte, V. Rousseau, L. Chebane, L. Ysebaert, V. Levy, J. L. Montastruc, F. Despas
FUNDAMENTAL & CLINICAL PHARMACOLOGY
(2021)
Correction
Hematology
C. Carpio, R. Bouabdallah, L. Ysebaert
