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
Yue Lu, Rui-Juan Sun, Jian-Ping Zhang, Fang Xu, Zhi-Cong Du, Ge-Le Tong, Yun Wang, Dao-Pei Lu
Summary: This study retrospectively analyzed the outcomes of 15 BPDCN patients who underwent allo-HSCT with myeloablative conditioning. The results showed that allo-HSCT with myeloablative conditioning is an effective option for BPDCN patients in complete remission, with most patients surviving after transplantation.
LEUKEMIA & LYMPHOMA
(2022)
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
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.
Article
Medicine, General & Internal
Jinzhi Chen, Xi Zhang, Linlin Ma, Yuan Gao, Zhanli Fu, Meng Liu
Summary: This article reports a case of a BPDCN patient who developed PTLD after allogeneic HSCT. The case report suggests that PTLD should be considered when new enlarged lymph nodes occur in BPDCN patients, and F-18-FDG PET/CT can provide additional evidence for further diagnosis.
FRONTIERS IN MEDICINE
(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
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)
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)
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)
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
Hematology
Koji Izutsu, Shinichi Makita, Kisato Nosaka, Makoto Yoshimitsu, Atae Utsunomiya, Shigeru Kusumoto, Satoko Morishima, Kunihiro Tsukasaki, Toyotaka Kawamata, Takaaki Ono, Shinya Rai, Hiroo Katsuya, Jun Ishikawa, Hironori Yamada, Kazunobu Kato, Masaya Tachibana, Yasuyuki Kakurai, Nobuaki Adachi, Kensei Tobinai, Kentaro Yonekura, Kenji Ishitsuka
Summary: This study evaluated the efficacy and safety of valemetostat, an inhibitor of EZH2 and EZH1, in treating relapsed or refractory ATL. The results showed that valemetostat demonstrated promising efficacy and tolerability in heavily pretreated patients with R/R ATL, warranting further investigation.
Review
Hematology
Tomohiro Aoki, Christian Steidl
Summary: The emergence and rapid development of single-cell technologies have transformed cancer research, providing powerful tools to understand cellular heterogeneity and interactions at the single-cell level. Recent advances in single-cell RNA sequencing have improved our understanding of lymphoma, particularly classical Hodgkin lymphoma (CHL), by revealing heterogeneity in malignant cell differentiation states and the composition of the tumor microenvironment (TME). These technologies hold promise for biomarker discovery and immunotherapeutic development for lymphoma treatment.
Article
Oncology
Takaaki Ono, Naoto Takahashi, Masahiro Kizaki, Tatsuya Kawaguchi, Ritsuro Suzuki, Kazuhito Yamamoto, Kazunori Ohnishi, Tomoki Naoe, Itaru Matsumura
Summary: Age and comorbidities should be taken into consideration when selecting TKIs for first-line treatment in CML-CP patients. This study found that the choice between imatinib and second-generation TKIs did not significantly impact treatment outcomes, regardless of age. However, older patients had lower overall survival rates compared to younger patients.
Review
Medicine, General & Internal
Chih-Yi Liu, Nai-Wen Kang, Kengo Takeuchi, Shih-Sung Chuang
Summary: Merkel cell carcinoma (MCC) is an aggressive and potentially metastatic rare primary neuroendocrine carcinoma (NEC) of the skin. Combined MCCs, which have a higher probability of metastasis, may be misdiagnosed due to small or superficial biopsy samples. This case report highlights the importance of timely diagnosis and appropriate management, as well as the presence of both MCC and non-MCC components in metastatic nodes.
Article
Cell Biology
Misayo Miyake, Seiji Sakata, Naoko Tsuyama, Yuki Togashi, Masaaki Noguchi, Nobuhiro Tsukada, Takashi Kumagai, Yuko Mishima, Kengo Takeuchi
Summary: This study presents the first report of nodal marginal zone lymphoma (NMZL) with tissue eosinophilia and highlights the distinctive morphological features and diagnostic factors for this type of lymphoma.
Article
Cell Biology
Kyoko Yamashita, Satoko Baba, Yuki Togashi, Akito Dobashi, Keisuke Ae, Seiichi Matsumoto, Miwa Tanaka, Takuro Nakamura, Kengo Takeuchi
Summary: The aim of this study was to clarify the genetic and pathological spectrum of AFST and determine whether histiocytic marker-positive cells are true neoplastic cells. The most frequent fusion gene in AFST was reported to be AHRR::NCOA2, with AHRR::NCOA3 potentially being the second most common fusion gene. Immunohistochemical analysis showed that CD163- and CD68-positive cells were diffusely distributed in all 12 cases, while varying levels of desmin-positive cells were observed in 9 cases. Double immunofluorescence staining and immunofluorescence in situ hybridisation confirmed the differences between CD163-positive cells and desmin-positive cells with AHRR::NCOA2 fusion in all 4 cases.
Article
Oncology
Michael L. Wang, Wojciech Jurczak, Pier Luigi Zinzani, Toby A. Eyre, Chan Y. Cheah, Chaitra S. Ujjani, Youngil Koh, Koji Izutsu, James N. Gerson, Ian Flinn, Benoit Tessoulin, Alvaro J. Alencar, Shuo Ma, David Lewis, Ewa Lech-Maranda, Joanna Rhodes, Krish Patel, Kami Maddocks, Nicole Lamanna, Yucai Wang, Constantine S. Tam, Talha Munir, Hirokazu Nagai, Francisco Hernandez-Ilizaliturri, Anita Kumar, Timothy S. Fenske, John F. Seymour, Andrew D. Zelenetz, Binoj Nair, Donald E. Tsai, Minna Balbas, Richard A. Walgren, Paolo Abada, Chunxiao Wang, Junjie Zhao, Anthony R. Mato, Nirav N. Shah
Summary: This study evaluated the safety and efficacy of Pirtobrutinib, a noncovalent BTK inhibitor, in patients with mantle-cell lymphoma who had previously received covalent BTK inhibitors. The results showed that Pirtobrutinib demonstrated durable efficacy and was well tolerated in this heavily pretreated patient population.
JOURNAL OF CLINICAL ONCOLOGY
(2023)
Editorial Material
Hematology
Kennosuke Karube, Kengo Takeuchi
Editorial Material
Hematology
Kentaro Narita, Kengo Takeuchi
Letter
Biophysics
Risa Koresawa-Shimizu, Ritsuro Suzuki, Yasufumi Uehara, Nobuhiro Hiramoto, Masashi Sawa, Takahiro Fukuda, Keisuke Kataoka, Yoshinobu Kanda, Tatsuo Oyake, Yasushi Kubota, Naoyuki Uchida, Shingo Yano, Hikaru Kobayashi, Junji Tanaka, Yoshiko Atsuta, Eisei Kondo
BONE MARROW TRANSPLANTATION
(2023)
Article
Oncology
Koji Izutsu, Takahiro Kumode, Junichiro Yuda, Hirokazu Nagai, Yuko Mishima, Youko Suehiro, Kazuhito Yamamoto, Tomoaki Fujisaki, Kenji Ishitsuka, Kenichi Ishizawa, Takayuki Ikezoe, Momoko Nishikori, Daigo Akahane, Jiro Fujita, Minh Dinh, David Soong, Hidehisa Noguchi, Jeppe Klint Buchbjerg, Elena Favaro, Noriko Fukuhara
Summary: This study presented the results of epcoritamab monotherapy in Japanese patients, showing durable treatment responses and manageable safety profile. The drug has potential therapeutic effects in relapsed or refractory diffuse large B-cell lymphoma.
Letter
Hematology
Koji Izutsu, Kazuhito Yamamoto, Koji Kato, Takayuki Ishikawa, Noriko Fukuhara, Yasuhito Terui, Ilseung Choi, Sumiko Okubo, Natsumi Ogawa, Mizu Sakai, Yasuko Nishimura, Brenda Chyla, Yan Sun, Dai Maruyama
INTERNATIONAL JOURNAL OF HEMATOLOGY
(2023)
Article
Medicine, General & Internal
Emiko Sugawara, Yasuyuki Shigematsu, Gulanbar Amori, Keisuke Sugita, Junji Yonese, Kengo Takeuchi, Kentaro Inamura
Summary: This study characterized the immunohistochemical and morphological characteristics of female urethral adenocarcinoma using 9 cases. Two subtypes of adenocarcinoma with distinct staining patterns were identified: the CDX2- and PAX8-expressing subtype and the clear cell carcinoma marker-expressing subtype. These findings are important for the accurate diagnosis of this rare tumor type.
Article
Oncology
Wataru Munakata, Koji Izutsu, Yuko Mishima, Hirokazu Nagai, Yuko Ishihara, Junji Suzumiya, Yuzuru Kanakura, Toshihiro Nanki, Takeshi Miyake, Atsuko Kawasaki, Tatsuya Yoshinaga, Kenichi Ishizawa
Summary: This study assessed the tolerability, safety, and pharmacokinetics of mosunetuzumab in Japanese patients with relapsed/refractory B-cell NHL. The results demonstrated that mosunetuzumab has acceptable safety and antitumor activity in Japanese patients, and the recommended Phase II dose was identified.
JAPANESE JOURNAL OF CLINICAL ONCOLOGY
(2023)
Article
Oncology
Carla Casulo, Armando Santoro, Guillaume Cartron, Kiyoshi Ando, Javier Munoz, Steven Le Gouill, Koji Izutsu, Simon Rule, Pieternella Lugtenburg, Jia Ruan, Luca Arcaini, Marie-Laure Casadebaig, Brian Fox, Nurgul Kilavuz, Nils Rettby, Justine Dell'Aringa, Lilia Taningco, Richard Delarue, Myron Czuczman, Thomas Witzig
Summary: The immune checkpoint inhibitor durvalumab shows potential as a strategy for enhancing immune responses and improving standard therapies in patients with hematologic malignancies. This study evaluated the safety and efficacy of durvalumab in combination with standard-of-care therapies for lymphoma or chronic lymphocytic leukemia (CLL) and found limited benefits of durvalumab monotherapy or combination therapy.
