Article
Multidisciplinary Sciences
Amanda N. Henning, Myagmarjav Budeebazar, Delgerbat Boldbaatar, Dahgwahdorj Yagaanbuyant, Davaadorj Duger, Khishigjargal Batsukh, Huizhi Zhou, Ryan Baumann, Robert D. Allison, Harvey J. Alter, Naranjargal Dashdorj, Valeria De Giorgi
Summary: Chronic HCV infection is linked to BNHL diseases, and RNA sequencing analysis reveals unique gene expression patterns associated with B cell transformation in both HCV and BNHL cases. Viral-mediated clonal expansion of anergic-like B cells and dysregulation of epigenetic regulatory genes are observed in HCV-associated BNHL, suggesting a potential mechanism for lymphoma development.
Review
Oncology
Roberto Castelli, Monica Balzarotti, Emanuele Salvi, Roberta Simona Rossi, Giorgio Lambertenghi Deliliers, Luigi Bergamaschini, Antonio Gidaro
Summary: Splenic marginal zone lymphoma (SMZL) is a rare lymphoproliferative disease affecting elderly patients. It primarily involves the peripheral blood, bone marrow, and spleen, while sparing the lymph nodes. The prolonged antigen stimulation of B lymphocytes and microenvironment play a crucial role in the development of SMZL. NOTCH2 and NFk-B signaling integration has been identified as the primary mechanism of neoplastic proliferation in SMZL. The prognosis of SMZL depends on individual risk factors and patients' health status.
Article
Hematology
Gabriela Bastidas-Mora, Silvia Bea, Alba Navarro, Eva Gine, Dolors Costa, Julio Delgado, Tycho Baumann, Laura Magnano, Alfredo Rivas-Delgado, Neus Villamor, Dolors Colomer, Monica Lopez-Guerra, Maria Rozman, Olga Balague, Daniel Martinez, Maria Joao Baptista, Lourdes Escoda, Miguel Alcoceba, Margarita Blanes, Fina Climent, Elias Campo, Andrew Wotherspoon, Armando Lopez-Guillermo, Estella Matutes
Summary: The study describes 36 patients with splenic marginal zone lymphoma (SMZL) with transformation, with predictors including cytopenias, hypoalbuminaemia, and complex karyotype. Patients with SMZL-T have a higher risk of death, mostly developing diffuse large B-cell lymphoma post transformation, with predictors for worse survival being high-risk International Prognostic Index and lack of complete response.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Andreea Sima, Peter Hollander, Eva Baecklund, Karin E. Smedby, Gunilla Enblad, Rose-Marie Amini
Summary: This study assessed the prevalence of viral hepatitis and AID in SMZL patients in a Swedish population and their treatment outcomes. The study found that upfront splenectomy was associated with better overall survival and progression-free survival. History of viral hepatitis and AID did not impact the outcomes of SMZL.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Medicine, Research & Experimental
Kei Nakayama, Takeshi Yoshida, Yoshiaki Nakayama, Noriaki Iguchi, Yuta Namba, Morichika Konishi, Hiroshi Hasegawa
Summary: This study focused on investigating the impact of malnutrition on the spleen, revealing significant reductions in spleen weight and red pulp ratio in dietary-restricted mice. It was found that macrophages in the marginal zone of the spleen play a crucial role in the dietary restriction-induced splenic involution.
Article
Hematology
Rui Lyu, Tingyu Wang, Yi Wang, Wenjie Xiong, Huijun Wang, Yuting Yan, Qi Wang, Wei Liu, Gang An, Wenyang Huang, Weiwei Sui, Yan Xu, Dehui Zou, Jianxiang Wang, Lugui Qiu, Shuhua Yi
Summary: This study found that undetectable minimal residual disease (uMRD) in splenic marginal zone lymphoma (SMZL) patients is an independent prognostic factor, helping to predict patient survival.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Review
Oncology
Juan Pablo Alderuccio, Izidore S. Lossos
Summary: NOTCH signaling, particularly NOTCH2, plays a critical role in the pathogenesis of splenic marginal zone lymphoma (SMZL). Frequent mutations of NOTCH2 in SMZL underscore its importance in the disease. While NOTCH signaling presents an appealing target for drug discovery in SMZL, previous efforts have not shown meaningful clinical benefit or faced safety concerns.
LEUKEMIA & LYMPHOMA
(2022)
Review
Pathology
Alberto Zamo, Michiel van den Brand, Fina Climent, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini, Siok-Bian Ng, Sarah L. Ondrejka, Leticia Quintanilla-Martinez, Lorinda Soma, Andrew Wotherspoon
Summary: Session 3 of the lymphoma workshop at the joint meeting of the European Association for Haematopathology and the Society for Hematopathology discussed splenic and nodal marginal zone lymphomas, transformation in marginal zone lymphomas, and pediatric nodal marginal zone lymphomas. The case review emphasized the challenges in diagnosing marginal zone lymphomas, including distinguishing between different types and the need for molecular studies to establish a final diagnosis. The workshop also addressed the overlapping spectrum between pediatric nodal marginal zone lymphoma and pediatric-type follicular lymphoma.
Article
Genetics & Heredity
Audrey N. Jajosky, Anna L. Mitchell, Mahmut Akgul, Shashirekha Shetty, Jennifer M. Yoest, Stanton L. Gerson, Navid Sadri, Kwadwo A. Oduro
Summary: Germline disruptive variants in the POT1 gene predispose individuals to multiple types of cancer. We report a case of splenic marginal zone lymphoma in a 65-year-old male with a germline POT1 variant. This likely pathogenic variant represents one of the most deleterious POT1 variants linked to familial cancer.
Article
Pathology
Simona Righi, Domenico Novero, Laura Godio, Clara Bertuzzi, Francesco Bacci, Claudio Agostinelli, Carlo Sagramoso, Maura Rossi, Milena Piccioli, Anna Gazzola, Claudia Mannu, Giovanna Roncador, Elena Sabattini
Summary: By studying multiple cases of LPL and SMZL, it was found that MNDA expression significantly supports the diagnosis of SMZL, especially in cases where MYD88 p.Leu265Pro mutation status and/or SMZL-related genetic aberrations are unavailable.
Article
Multidisciplinary Sciences
Nathalie Diener, Jean -Fred Fontaine, Matthias Klein, Thomas Hieronymus, Florian Wanke, Florian C. Kurschus, Andreas Ludwig, Carl Ware, Paul Saftig, Tobias Bopp, Bjoern E. Clausen, Ronald A. Backer
Summary: This study identifies ADAM10 as a crucial regulator of cDC1 and cDC2 homeostasis in the splenic marginal zone, affecting their commitment, differentiation, and survival. ADAM10 deficiency leads to a loss of specific cDC subsets but also induces the emergence of other subsets, regulating the transcriptional programming, turnover, and biology of cDC and MMM.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Immunology
Lingling Wang, Qian Huang, Jiao Tang, Jun Feng, Yongfen Huang, Jianming Dong, Yuexin Cheng, Hao Xu, Yuqing Miao
Summary: Nodal Marginal Zone Lymphoma (NMZL) is a rare form of lymphoma that can be difficult to differentiate from Lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia (LPL/WM). This case report highlights the first case of NMZL with elevated monoclonal immunoglobulin M (IgM) complicated by atypical nontraumatic chylothorax. It emphasizes the importance of considering hematologic malignancies in patients with atypical nontraumatic chylothorax.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Ana Muntanola, Maria Teresa Villalobos, Sonia Gonzalez-Villambrosia, Maria Jose Rodriguez-Salazar, Ana Jimenez-Ubieto, Gabriela Bastidas-Mora, Raul Cordoba, Maria Infante, Maria Jesus Vidal, Francisco Javier Diaz, Monica Baile, Mariana Bastos-Oreiro, Carlos Panizo, Juan Manuel Sancho, Belen Navarro, Tomas Garcia, Lourdes Escoda, Pau Abrisqueta, Maria Jose Terol, Raquel de Campo, Pablo Mozas, Armando Lopez-Guillermo, Antonio Salar, Carlos Montalban
Summary: The aim of this study was to analyze compliance with the 2014 GELTAMO SMZL Guidelines and evaluate the outcome of splenic marginal zone lymphoma (SMZL) patients. A prospective multicenter study was conducted on 181 SMZL patients diagnosed between 2014 and 2020. The study found that 57% of patients followed the Guidelines, and the overall response rate was higher in the rituximab chemotherapy and rituximab arms compared to the splenectomy arm. The 5-year overall survival rate was 77% and the 5-year lymphoma-specific survival rate was 93%.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Immunology
Rui Tada, Koichiro Nagao, Riki Tanaka, Sumire Yamada, Ayano Watanabe, Yoichi Negishi
Summary: Autoimmune diseases are difficult to treat, but improving apoptotic cell processing could be a potential strategy. Liposomes composed of phosphatidylserine (PS) have shown anti-inflammatory effects and could be used as antigen delivery vesicles to target autoimmune diseases.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2022)
Article
Medicine, General & Internal
Leonor Silva, Mafalda Alpoim, Ana Ribeiro, Pedro Caiano Gil, Rute Lopes Cacola
Summary: Splenic marginal zone lymphoma (SMZL) is a rare type of lymphoma, with almost all patients having bone marrow involvement, but only one-third having liver involvement. Some patients have a history of hepatitis C virus infection, which may be related to the development of lymphoma.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Letter
Oncology
Marzia Varettoni, Silvia Zibellini, Michele Merli, Daniela Drandi, Cristina Jimenez, Daniela Furlan, Virginia Valeria Ferretti, Nicole Fabbri, Irene Dogliotti, Chiara Varraso, Martina Ferrante, Emilia Cappello, Veronica Peri, Chiara Cavalloni, Michela Borriero, Giulia Vittoria Facchetti, Simone Ferrero, Luca Arcaini, Ramon Garcia-Sanz
HEMATOLOGICAL ONCOLOGY
(2023)
Review
Oncology
Francesco Passamonti, Emanuele Nicastri, Alice Di Rocco, Attilio Guarini, Adalberto Ibatici, Stefano Luminari, Malgorzata Mikulska, Carlo Visco
Summary: This review provides the latest data on patients with lymphoma and COVID-19, and discusses practical guidance from diagnosis to follow-up. Lymphoma patients are at a higher risk of severe COVID-19 and may not be fully protected by vaccination. Passive immunization with monoclonal antibodies and antiviral drugs have shown effectiveness in managing lymphoma patients with COVID-19.
HEMATOLOGICAL ONCOLOGY
(2023)
Article
Hematology
Christian Pecquet, Nicolas Papadopoulos, Thomas Balligand, Ilyas Chachoua, Amandine Tisserand, Audrey Nedelec, Didier Vertommen, Anita Roy, Caroline Marty, Harini Nivarthi, Mira El-Khoury, Eva Hug, Andrea Majoros, Erica Xu, Oleh Zagrijtschuk, Tudor E. Fertig, Daciana S. Marta, Heinz Gisslinger, Bettina Gisslinger, Martin Schalling, Ilaria Casetti, Elisa Rumi, Daniela Pietra, Chiara Cavalloni, Luca Arcaini, Mario Cazzola, Norio Komatsu, Yoshihiko Kihara, Yoshitaka Sunami, Yoko Edahiro, Marito Araki, Roman Lesyk, Veronika Buxhofer-Ausch, Sonja Heibl, Florence Pasquier, Violaine Havelange, Isabell Plo, William Vainchenker, Robert Kralovics, Stefan N. Constantinescu
Summary: Mutant CALR proteins bind to and activate the TpoR in cells, driving the development of myeloproliferative neoplasms. These mutant CALR proteins can be found in patient plasma complexed with sTFR1, which increases their stability. They can specifically interact with TpoR on target cells and promote thrombopoietin-independent colony formation.
Article
Hematology
Annalisa Arcari, Valentina Tabanelli, Francesco Merli, Luigi Marcheselli, Michele Merli, Monica Balzarotti, Vittorio Ruggero Zilioli, Alberto Fabbri, Federica Cavallo, Gloria Margiotta Casaluci, Alessandra Tucci, Benedetta Puccini, Elsa Pennese, Alice Di Rocco, Manuela Zanni, Leonardo Flenghi, Guido Gini, Roberto Sartori, Annalisa Chiappella, Sara Veronica Usai, Monica Tani, Dario Marino, Luca Arcaini, Daniele Vallisa, Michele Spina
Summary: Up to 10%-15% of DLBCL cases in elderly patients are related to HCV infection. HCV+ patients were older, less fit, and had frequent extranodal involvement compared to HCV- patients. The use of DAAs in HCV+ patients showed good tolerance and a positive impact on progression-free survival.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Oncology
Chiara Rossi, Sara Fraticelli, Marianna Fanizza, Alberta Ferrari, Elisa Ferraris, Alessia Messina, Angelica Della Valle, Chiara Annunziata Pasqualina Anghelone, Angioletta Lasagna, Gianpiero Rizzo, Lorenzo Perrone, Maria Grazia Sommaruga, Giulia Meloni, Silvia Dallavalle, Elisabetta Bonzano, Marco Paulli, Giuseppe Di Giulio, Adele Sgarella, Marco Lucioni
Summary: Accurate evaluation of breast cancer on bioptic samples is crucial for guiding therapeutic decisions. This study assessed the concordance of hormone receptors and Ki-67 between biopsy and surgical specimens. The results suggest suboptimal concordance for ER-low-positive, c-erbB2/HER2, and Ki-67, indicating the need for further training in this area.
BREAST CANCER RESEARCH AND TREATMENT
(2023)
Review
Oncology
Pier Luigi Zinzani, Francesca Romana Mauro, Alessandra Tedeschi, Marzia Varettoni, Francesco Zaja, Giovanni Barosi
Summary: Zanubrutinib has been approved for the treatment of various lymphoproliferative disorders, providing a significant breakthrough for patients who are resistant or relapse after recommended therapies. Due to the lack of systematic studies and comparative randomized clinical trials, the optimal use of zanubrutinib in approved indications presents challenges, especially in earlier stages of the disorders. This article presents the results of expert panel discussions aimed at identifying unmet clinical needs and proposing recommendations for the management of these needs.
HEMATOLOGICAL ONCOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Sara Fraticelli, Marco Lucioni, Giuseppe Neri, Deborah Marchiori, Caterina Cristinelli, Michele Merli, Rodolfo Monaco, Tiziana Borra, Antonio Lazzaro, Silvia Uccella, Luca Arcaini, Marco Paulli
Summary: Castleman disease (CD) is a rare lymphoproliferative disorder with various clinico-pathological subtypes. The pathogenesis of CD is still uncertain, but alterations in T-cell subsets may contribute to the development of CD. In this study, the distribution of T-cell subsets in the clinico-pathological spectrum of CD was investigated. The results showed decreased CD4/CD8 ratio and lower number of FOXP3+ T-reg cells in CD cases compared to non-specific reactive lymph nodes.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
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
Marco Lucioni, Sara Fraticelli, Giovanni Santacroce, Arturo Bonometti, Nicola Aronico, Roberta Sciarra, Marco Vincenzo Lenti, Paola Ilaria Bianchi, Giuseppe Neri, Monica Feltri, Benedetto Neri, Giuseppina Ferrario, Roberta Riboni, Gino Roberto Corazza, Alessandro Vanoli, Luca Arcaini, Marco Paulli, Antonio Di Sabatino
Summary: This retrospective study aimed to describe the clinical-pathological characteristics of intestinal T-cell lymphomas (ITCL) and possibly identify their hallmarks. The study included 28 patients, with most cases associated with celiac disease. The presence of CD, peripheral lymphocytosis, advanced Lugano clinical stage, and the histological subtype ITCL-NOS were significantly associated with worse survival.
Article
Pharmacology & Pharmacy
Simona De Gregori, Eleonora Gelli, Mara Capone, Giulia Gambini, Elisa Roncoroni, Marianna Rossi, Claudia Patricia Tobar Cabrera, Gianluca Martini, Ludovica Calabretta, Luca Arcaini, Riccardo Albertini, Patrizia Zappasodi
Summary: The combination of hypomethylating agents (HMA) azacytidine or decitabine with venetoclax (VEN) has been approved by the FDA for the treatment of acute myeloid leukemia (AML) patients aged over 75 years and those unsuitable for intensive chemotherapy. To prevent fungal infection during the early phase of treatment, posaconazole (PCZ) is commonly administered as primary prophylaxis. 165 plasma samples from 11 elderly AML patients receiving combined treatment with HMA, VEN, and PCZ were analyzed to determine the serum levels of venetoclax and the need for therapeutic drug monitoring due to high inter-individual variability in pharmacokinetics.
Article
Microbiology
Valentina Zuccaro, Greta Petazzoni, Irene Mileto, Marta Corbella, Erika Asperges, Paolo Sacchi, Sara Rattotti, Marzia Varettoni, Irene Defrancesco, Patrizia Cambieri, Fausto Baldanti, Luca Arcaini, Raffaele Bruno
Summary: Several studies have shown a strong connection between gut microbiota and the response to immunotherapy in tumor patients, suggesting that gut microbiota can serve as a biomarker for response. This study aimed to compare the gut microbiota diversity in chronic lymphocytic leukemia patients treated with B-cell receptor inhibitors for at least 12 months. The findings revealed differences in bacterial distribution between different response groups.
Editorial Material
Hematology
Michele Merli, Francesco Passamonti, Luca Arcaini
Summary: Recent clinical studies have shown that patients with Hepatitis C virus-associated indolent lymphoproliferative disorders (LPD) can achieve lymphoma responses when treated solely with direct-acting antivirals (DAAs). However, the molecular mechanisms underlying LPD responses to DAAs are not well understood. In this paper, the authors provide new molecular insights, reporting intraclonal diversification and persistence of B-cell clones in most cases, even after viral eradication and positive clinical outcomes. These findings suggest that achieving a molecular response may not be necessary for a 'functional cure' in these patients. Further immunogenetic and mutational studies are needed to better understand this biological puzzle and refine treatment strategies.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Editorial Material
Hematology
Francesco Passamonti, Alessandra Bandera
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Oncology
Francesco Passamonti, Youbei Lou, Manoj Chevli, Pranav Abraham
Summary: This observational study evaluated the outcomes of US myelofibrosis patients who received ruxolitinib treatment, and found that subsequent treatment with fedratinib led to improved survival compared to non-fedratinib treatment.
Article
Hematology
Fanbi Meng, Shuang Chen, Chunliang Liu, Muhammad Shoaib Khan, Yan Yan, Jun Wan, Yue Xia, Chenglin Sun, Mengnan Yang, Renping Hu, Kesheng Dai
Summary: PKC plays a critical role in regulating megakaryocyte development and platelet production in thrombocytopenia. The inhibition of PKC activation delays and reduces radiation-induced megakaryocyte apoptosis, leading to improved platelet count recovery. This study provides a potential therapeutic target for treating thrombocytopenia.
BLOOD CELLS MOLECULES AND DISEASES
(2024)
Article
Hematology
Xia Chen, Fang Liu, Yuanyuan Ren, Luyang Zhang, Yang Wan, Wenyu Yang, Xiaojuan Chen, Li Zhang, Yao Zou, Yumei Chen, Xiaofan Zhu, Ye Guo
Summary: Unrelated umbilical cord blood transplantation (UCBT) is an effective alternative treatment for pediatric patients with bone marrow failure. The use of the TBI/BU + FLU + CY conditioning regimen ensures a high engraftment rate for UCBT and overcomes the challenge of graft failure. Secondary salvage use of cord blood transplantation may still be useful for patients who have failed after other transplantation.
BLOOD CELLS MOLECULES AND DISEASES
(2024)
Article
Hematology
Chithra D. Palani, Xingguo Zhu, Manickam Alagar, Otis C. Attucks, Betty S. Pace
Summary: This study evaluated the ability of a novel Bach1 inhibitor, HPP-D, to induce HbF in KU812 cells and primary sickle erythroid progenitors. The results showed that HPP-D increased HbF levels and decreased Bach1 protein levels in both cell types. Furthermore, it enhanced NRF2 binding to the gamma-globin promoter antioxidant response elements, leading to an open chromatin configuration and increased gamma-globin transcription.
BLOOD CELLS MOLECULES AND DISEASES
(2024)
Letter
Hematology
Marcos Borato Viana, Erica Louback Oliveira, Andre Rolim Belisario
BLOOD CELLS MOLECULES AND DISEASES
(2024)
Article
Hematology
Ke Zhang, Longying Ye, Yanhui Jin, Yuan Chen, Shuting Jiang, Haixiao Xie, Lihong Yang, Mingshan Wang
Summary: This study investigated two pedigrees with FV deficiency and identified three mutations that may have deleterious effects on the function and structure of FV.
BLOOD CELLS MOLECULES AND DISEASES
(2024)