Article
Oncology
Wei Song, Haixi Zhang, Fan Yang, Kiichi Nakahira, Cheng Wang, Keqian Shi, Ruoyu Zhang
Summary: In this study, the researchers generated the first single cell landscape for hepatosplenic T-cell lymphoma (HSTCL) and characterized the molecular pathogenesis underlying the disease progression. They found unique gene expressing signatures in malingnant gamma delta T cells of HSTCL and discovered two transcriptionally distinct tumor subtypes during the disease progression.
Article
Oncology
Qian Wang, Yibin Jiang, Qian Zhu, Yishan Duan, Xiaochen Chen, Ting Xu, Zhengming Jin, Caixia Li, Depei Wu, Haiwen Huang
Summary: HSTCL typically presents with splenomegaly, hepatomegaly, and B symptoms. Some patients respond to chemotherapy, with those receiving allogeneic hematopoietic cell transplantation having a survival time ranging from 10 to 27 months. Intensive induction chemotherapy followed by HSCT may improve the prognosis of HSTCL.
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
(2021)
Article
Biology
Vivian Hathuc, Friederike Kreisel
Summary: This review discusses the genetic landscape of peripheral T-cell lymphoma (PTCL) and its role in subclassification, prognosis, and potential targeted therapy. The article emphasizes the use of a multiparameter approach in the diagnosis and classification, and introduces the latest applications of gene expression profiling and next-generation sequencing (NGS) techniques in the pathobiology of PTCL.
Article
Hematology
Youngkyung Jeon, Sang Eun Yoon, Junhun Cho, Seok Jin Kim, Won Seog Kim
Summary: Hepatosplenic T cell lymphoma (HSTCL) is a rare and aggressive lymphoma with no standard treatment and poor treatment response. Most patients present with B symptoms, hepatomegaly, and splenomegaly. In general, HSTCL has a poor prognosis and requires further research on genetic and biological information.
ANNALS OF HEMATOLOGY
(2023)
Article
Medicine, General & Internal
Yajun Li, Kailin Chen, Chaohui Zuo, Ruolan Zeng, Yizi He, Xiaoyan Chen, Ling Xiao, Hui Zhou
Summary: This study retrospectively reviewed 123 patients with HSTCL diagnosed between 1975 and 2016 in the SEER database, and found that young patients and a strong male predominance were more common in HSTCL. The overall survival of HSTCL patients was similar to PTCL-NOS patients, but worse than ALK+ ALCL patients, and chemotherapy combined with topical treatment may improve survival rates. Further studies are needed to better understand and treat this rare and unfavorable disease.
INTERNATIONAL JOURNAL OF GENERAL MEDICINE
(2021)
Review
Oncology
Dominique Bron, Laurence De Leval, Sandra Michiels, Sebastian Wittnebel
Summary: Hepatosplenic lymphoma (HSTCL) is a rare T-cell malignancy with poor survival in young males, but recent studies suggest that allogeneic transplantation and specific chemotherapy may be effective treatment options. Recent molecular studies have identified potential targets for new therapeutic strategies.
CURRENT OPINION IN ONCOLOGY
(2021)
Article
Biology
Hyunsung Kim, Young Hyeh Ko
Summary: Extranodal NK/T-cell lymphoma is a tumor that occurs in extranodal sites, most commonly in the nasal cavity. It is associated with the presence of the EBV genome, specific immunophenotypes, and mutations in certain pathways and genes. Understanding the molecular pathogenesis of ENKTL and developing better treatment strategies are important for managing this disease.
Article
Cell & Tissue Engineering
Xueer Xu, Cheng Zu, Mingming Zhang, Pingnan Xiao, Ruimin Hong, Jingjing Feng, Huijun Xu, Jiazhen Cui, Jian Yu, Jimin Shi, Guoqing Wei, Alex H. Chang, He Huang, Yongxian Hu
Summary: This study reported on a patient with refractory hepatosplenic & gamma;& delta; T-cell lymphoma who achieved complete remission and maintained it after receiving HLA fully-mismatched sibling-derived CD7 CAR-T therapy followed by haploidentical hematopoietic stem cell transplantation. This is the first case of its kind, highlighting the potential of CAR-T therapy and haploidentical HSCT in treating relapsed/refractory T-cell lymphoma.
CELL TRANSPLANTATION
(2023)
Article
Hematology
Yue-Hua Zhang, Zheng Li, Sha Zhao, Wen-Yan Zhang, Qing-Lin Liu, Wei-Ping Liu, Li-Min Gao
Summary: This study retrospectively investigated the clinicopathologic features of seven cases of extranodal natural killer/T-cell lymphoma (ENKTL) with hepatosplenic involvement. The study found that ENKTL with hepatosplenic involvement is rare and poorly understood. However, there are two histopathologic patterns and L-asparaginase-based chemotherapy might be effective.
ANNALS OF HEMATOLOGY
(2023)
Article
Hematology
Wenqiu Huang, Song Xue, Yongping Zhang, Fuhong Liu, Min Tian, Yanping Wang, Fang Li, Yiyan Lu, Jingbo Wang
Summary: This study suggests that salvage allogeneic hematopoietic stem cell transplantation (allo-HSCT) in combination with enhanced myeloablative preconditioning may be an effective and safe treatment for refractory liver and spleen T-cell lymphomas.
ANNALS OF HEMATOLOGY
(2023)
Article
Oncology
Ginevra Lolli, Beatrice Casadei, Vittorio Stefoni, Lisa Argnani, Francesca Bonifazi, Pier Luigi Zinzani
Summary: Hepatosplenic T-cell lymphoma is a challenging lymphoma to treat, but tandem consolidation therapy with auto-stem cell transplant and allo-stem cell transplant may induce a lasting response and potential cure.
Article
Oncology
Leyla Bojanini, Liuyan Jiang, Alexander J. Tun, Ernesto Ayala, David M. Menke, Bradford Hoppe, Mohamed A. Kharfan-Dabaja, Han W. Tun, Muhamad Alhaj Moustafa
Summary: Hepatosplenic T-cell lymphoma is a rare and aggressive subtype of peripheral T-cell lymphoma with poor prognosis. Liver involvement and chronic immunosuppression are associated with worse survival outcomes in patients with HSTCL. Although splenectomy and allogeneic hematopoietic cell transplant have anecdotal benefit, larger studies are needed to determine their effectiveness.
CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
(2021)
Article
Oncology
Khaliqur Rahman, Tanvi Gupta, Ruchi Gupta, Lakshita Singh, Dinesh Chandra, Manoj Sarkar, Manish Singh, Sanjeev Kumar, Soniya Nityanand
Summary: This study investigated the prevalence and clinicopathological spectrum of hepatosplenic T-cell lymphoma (HSTCL) in the Indian subcontinent. The results showed that leukocytosis and a blastoid morphology were common in HSTCL, and most patients died within a year of diagnosis.
JOURNAL OF CANCER RESEARCH AND THERAPEUTICS
(2022)
Article
Genetics & Heredity
Vladimir Vasku, Jan Machal, Filip Zlamal, Anna Vasku
Summary: This study evaluated the survival of CTCL patients and analyzed 19 gene variants. The results showed that the TT genotype at position 2677 of the MDR1 gene is associated with longer survival in CTCL patients.
Review
Oncology
Wern Lynn Ng, Stephen M. Ansell, Patrizia Mondello
Summary: Current therapies for lymphoma primarily target tumor cells and pay less attention to the tumor microenvironment (TME). However, the TME plays a crucial role in the growth and survival of lymphoma. Therefore, targeting the TME has emerged as a new therapeutic strategy to comprehensively manage lymphoma and improve patient survival.
JOURNAL OF EXPERIMENTAL & CLINICAL CANCER RESEARCH
(2022)
Review
Pathology
Andrew L. Feldman, Camille Laurent, Marina Narbaitz, Shigeo Nakamura, Wing C. Chan, Laurence de Leval, Philippe Gaulard
Summary: This article discusses the classification and diagnostic evaluation of nodal T- and NK-cell lymphomas based on the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (2022 ICC). It highlights the grouping of T-follicular helper cell lymphoma into a single entity with three subtypes, as well as the genetic subtype of ALK-negative anaplastic large cell lymphoma (ALCL) associated with DUSP22 rearrangement. The introduction of a new provisional entity, primary nodal EBV-positive T-/NK-cell lymphoma, is also mentioned, along with evolving molecular data indicating distinct subgroups within PTCL, NOS.
Article
Gastroenterology & Hepatology
Vincent Wai-Hin Yuen, David Kung-Chun Chiu, Cheuk-Ting Law, Jacinth Wing-Sum Cheu, Cerise Yuen-Ki Chan, Bowie Po-Yee Wong, Chi-Ching Goh, Misty Shuo Zhang, Helen Do-Gai Xue, Aki Pui-Wah Tse, Yan Zhang, Henry Yee-Hin Lau, Derek Lee, Rex K. H. Au-Yeung, Chun-Ming Wong, Carmen Chak-Lui
Summary: This study aims to investigate how genetic composition and specific oncogenic pathways regulate the immune composition of hepatocellular carcinoma (HCC) and affect the response to immune checkpoint inhibitors (ICIs). By establishing mouse models with genetic genotypes similar to human HCCs using genome-editing techniques, the researchers found that "hot tumors" driven by genetic mutations responded well to anti-PD-1 treatment, while "cold tumors" did not. In addition, they discovered that the TKI sorafenib can enhance the sensitivity of "cold tumors" caused by certain genetic mutations to anti-PD-1 treatment.
JOURNAL OF HEPATOLOGY
(2023)
Article
Pathology
Come Sesboue, Jean Galtier, Marie Jeanneau, Annick Chauvel, Elodie Laharanne, Samuel Amintas, Jean-Philippe Merlio, Krimo Bouabdallah, Francois-Xavier Gros, Laurence de Leval, Audrey Gros, Marie Parrens
Summary: Distinguishing FL from NMZL can be difficult in cases with unusual morphologic and phenotypic features and without characteristic cytogenetic rearrangements. Ancillary techniques, such as FISH, RT-MLPA, and NGS, can provide diagnostic contributions for unclassified tumors. In this study, FISH detected 1p36 deletion in some FLs and unclassified tumors, while RT-MLPA signatures were associated with FL and NMZL subtypes. Combining RT-MLPA and NGS findings successfully discriminated most unclassified tumors towards FL or NMZL diagnosis.
Article
Pathology
Rita Sarkis, Olivier Burri, Claire Royer-Chardon, Frederica Schyrr, Sophie Blum, Mariangela Costanza, Stephane Cherix, Nathalie Piazzon, Carmen Barcena, Bettina Bisig, Valentina Nardi, Rossella Sarro, Giovanna Ambrosini, Martin Weigert, Olivier Spertini, Sabine Blum, Bart Deplancke, Arne Seitz, Laurence de Leval, Olaia Naveiras
Summary: Bone marrow cellularity assessment is crucial in evaluating bone marrow biopsies for hematologic and nonhematologic disorders. MarrowQuant 2.0 is an efficient digital hematopathology workflow that accurately quantifies the cellularity of human bone marrow biopsies by segmenting different compartments. This tool can be used for clinical research to explore biomarkers related to bone marrow stromal components.
Article
Biochemistry & Molecular Biology
Jennifer H. Kong, Claudia G. Vasquez, Sweta Agrawal, Prerna Malaney, Maria M. Mikedis, Andrea B. Moffitt, Lexy von Diezmann, Christina M. Termini
Summary: The process of evaluating and negotiating a tenure-track job offer in the life sciences lacks structure and consistency, leading to potential bias and unfair outcomes. To address this issue, we provide an overview of common aspects and recommendations for negotiating academic job offers to promote equitable recruitment of diverse faculty.
TRENDS IN BIOCHEMICAL SCIENCES
(2023)
Review
Pathology
Laurence de Leval, Andrew L. Feldman, Stefano Pileri, Shigeo Nakamura, Philippe Gaulard
Summary: Non-cutaneous extranodal NK/T cell lymphoproliferations are a rare group of tumors that primarily occur in the gastrointestinal tract, nasal area, spleen, and liver. Accurate diagnosis is crucial due to the diverse clinical presentations and similarities with other diseases.
Article
Oncology
Xiaomei Zhao, Biao Wang, Yuan Zhuang, Shisuo Du, Zhaochong Zeng
Summary: This study investigates the immunomodulatory effects of different dose-fractionation schedules and finds that a single high-dose radiation schedule has a stronger antitumor effect. The study also reveals the involvement of the cGAS/STING signaling pathway in the immunomodulatory effects of single high-dose radiation.
INTERNATIONAL JOURNAL OF RADIATION ONCOLOGY BIOLOGY PHYSICS
(2023)
Article
Oncology
Elisabeth Gross, Ralf-Axel Hilger, Franziska Lea Schuemann, Marcus Bauer, Alyssa Bouska, Christian Rohde, Edith Willscher, Jana Luetzkendorf, Lutz Peter Mueller, Bayram Edemir, Thomas Mueller, Marco Herling, Mascha Binder, Claudia Wickenhauser, Javeed Iqbal, Guido Posern, Thomas Weber
Summary: Histone lysine methyltransferase EZH2 is frequently altered in lymphoid tumors, and its overexpression or mutation is associated with tumor progression and resistance to chemotherapy. In this study, the combination treatment with SAM-competitive EZH2 inhibitors led to platinum resistance due to increased platinum efflux. Off-target effects of the inhibitors resulted in the upregulation of proteins associated with chemotherapy resistance.
Article
Oncology
Moritz Otte, Johanna Stachelscheid, Markus Glass, Linus Wahnschaffe, Qu Jiang, Waseem Lone, Aleksandr Ianevski, Tero Aittokallio, Javeed Iqbal, Michael Hallek, Stefan Huettelmaier, Alexandra Schrader, Till Braun, Marco Herling
Summary: T-prolymphocytic leukemia (T-PLL) is a rare and poor-prognostic mature T-cell leukemia. Elevated expression of miR-141/200c cluster in T-PLL cells promotes cell proliferation and alters the expression of genes involved in cell survival and differentiation. Low expression of STAT4, a target gene of miR-141/200c, is associated with an immature phenotype of T-PLL cells and shortened overall survival of T-PLL patients. This study reveals the oncogenic role of miR-141/200c-STAT4 signaling pathway in T-PLL, shedding light on the involvement of non-protein-coding genes in the pathogenesis of this devastating disease.
Meeting Abstract
Oncology
Asma Beldi-Ferchiou, Oro Saskia, Valerie Molinier-Frenkel, Francois Lemonnier, Allan Thiolat, Caroline Pilon, Jerome Giustiniani, Philippe Gaulard, Marie-Helene Delfau-Larue, Nicolas Ortonne, Jose L. Cohen
EUROPEAN JOURNAL OF CANCER
(2023)
Article
Oncology
Andrew M. Evens, Kristina S. Yu, Nicholas Liu, Andy Surinach, Katherine Holmes, Carlos Flores, Michelle A. Fanale, Darcy R. Flora, Susan K. Parsons
Summary: This study aimed to understand the treatment preferences and decision-making of US physicians when it comes to frontline treatment for stage III/IV classic Hodgkin lymphoma (cHL). Results showed that the most common factors considered by physicians when selecting 1L cHL treatments were trial efficacy/safety data and national guidelines. Although treatment selection varied based on patient characteristics, overall survival (OS) was consistently cited as the most important attribute.
Review
Pathology
Leticia Quintanilla-Martinez, Camille Laurent, Lorinda Soma, Siok-Bian Ng, Fina Climent, Sarah L. L. Ondrejka, Alberto Zamo, Andrew Wotherspoon, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini
Summary: The 2022 European Association for Haematopathology/Society for Hematopathology workshop in Florence, Italy discussed emerging entities and molecular subgroups in large B-cell lymphomas (LBCLs). The session focused on newly recognized diseases and their diagnostic challenges. Topics included high-grade/large B-cell lymphoma with 11q aberration (HG/LBCL-11q), LBCL with IRF4 rearrangement (LBCL-IRF4), primary bone diffuse large B-cell lymphoma (PB-DLBCL), and B-acute lymphoblastic leukemia (B-ALL) with IGH::MYC translocation.
Review
Pathology
Fina Climent, Alina Nicolae, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini, Sarah L. Ondrejka, Lorinda Soma, Andrew Wotherspoon, Alberto Zamo, Leticia Quintanilla-Martinez, Siok-Bian Ng
Summary: The 2022 European Association for Haematopathology/Society for Hematopathology lymphoma workshop discussed cytotoxic peripheral T-cell lymphomas and EBV-positive T/NK-cell lymphoproliferative diseases. Primary nodal-EBV-TNKL is a newly recognized entity associated with underlying immune deficiency/immune dysregulation. cPTCL-NOS demonstrates the TBX21 phenotype and shows an unexpected association with TFH lymphomas/LPDs.
Article
Immunology
Ariana Mihai, Sumedha Roy, Michael S. Krangel, Yuan Zhuang
Summary: The V(D)J recombination of antigen receptor loci is a highly regulated process in lymphocyte development. The recombination of Tcra gene occurs in CD4(+)CD8(+) double positive thymocytes and requires the Tcra enhancer (E & alpha;). E proteins, known regulators of DP thymocyte development, have three binding sites in E & alpha;. Mutants lacking one or two of the binding sites showed partial blockage at the positive selection stage of & alpha;& beta; T cell development, loss of germline transcription, and dysregulated gene rearrangement. The binding of E proteins to E & alpha; promotes Tcra repertoire diversity.
FRONTIERS IN IMMUNOLOGY
(2023)
Meeting Abstract
Oncology
Terry Tin Wai Chow, Wilson Yau Ki Chan, Ka Ho Chiu, Cheng Man, Hin Mark, Chak Ho Li, Ka Fai To, Chit Chow, Rex Kwok Him Au-Yeung, Elaine Tin Yan Cheung, Chi Kong Li
PEDIATRIC BLOOD & CANCER
(2023)