Article
Chemistry, Medicinal
Hana Votavova, Zuzana Urbanova, David Kundrat, Michaela Dostalova Merkerova, Martin Vostry, Monika Hruba, Jaroslav Cermak, Monika Belickova
Summary: DFX improves hematopoiesis in anemic myelodysplastic syndrome patients by modulating immune response, suppressing negative regulators of blood cell differentiation, and upregulating heme metabolism.
Article
Oncology
Mathias Schneeweiss-Gleixner, Georg Greiner, Susanne Herndlhofer, Julia Schellnegger, Maria-Theresa Krauth, Karoline Gleixner, Friedrich Wimazal, Corinna Steinhauser, Michael Kundi, Renate Thalhammer, Ilse Schwarzinger, Gregor Hoermann, Harald Esterbauer, Manuela Fodinger, Peter Valent, Wolfgang R. Sperr
Summary: Although iron overload is a clinical challenge, little is known about the clinical impact of HFE-variants in myelodysplastic syndromes. The study found that HFE-variants H63D and C282Y are frequently detected in Austrian MDS patients, who have higher ferritin levels at diagnosis, accumulate iron slightly faster, and have a better progression-free survival than non-mutated patients.
AMERICAN JOURNAL OF CANCER RESEARCH
(2021)
Article
Medicine, General & Internal
Felicitas Schulz, Ulrich Hauch, Sandra Ketzler-Henkel, Eyck von der Heyde, Michael Koenigsmann, Michael Lauseker, Nora Schulte, Ulrich Germing
Summary: Myelodysplastic syndromes and myeloproliferative neoplasms are hematologic diseases associated with bone marrow failure, and transfusion of red blood cells is necessary but leads to iron overload. Therefore, iron chelation therapy plays a crucial role in anemic patients.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Chemistry, Medicinal
Mengdie Su, Yanjia Zhao, Mei Li, Chenyang Jia, He Liu, Yue Zhang, Weiwei Li, Ying Peng, Jiang Zheng
Summary: This study identified two hydroxylated metabolites of Deferasirox (DFS) in rat liver microsomes, which were also detected in bile and urine. CYP1A2 and CYP3A4 were found to be the main enzymes responsible for the metabolic activation of DFS. DFS exhibited cytotoxicity towards primary hepatocytes, but pretreatment with ketoconazole and 1-aminobenzotrizole reduced its toxicity.
CHEMICAL RESEARCH IN TOXICOLOGY
(2023)
Article
Biology
Alkistis Adramerina, Nikoleta Printza, Emmanouel Hatzipantelis, Symeon Symeonidis, Labib Tarazi, Aikaterini Teli, Marina Economou
Summary: Thalassemia is a hereditary anemia that requires regular blood transfusions and chelation therapy. The new deferasirox film-coated tablet (DFX FCT) offers a more convenient and well-tolerated treatment option, although it may lead to increased adverse events in younger patients. The study confirmed the safety of DFX FCT in older children, but higher doses were required for effective chelation.
Article
Oncology
Laurent Pascal
Summary: Iron overload is harmful to elderly patients with low-risk myelodysplastic syndromes (MDS), especially those with cardiovascular disease. A randomized study has shown improved event-free survival with Iron Chelation Therapy (ICT) in these patients. Further research is needed to better understand the benefits of ICT.
BULLETIN DU CANCER
(2023)
Article
Hematology
Vladimir Riabov, Maximilian Mossner, Alexandra Stoehr, Johann-Christoph Jann, Alexander Streuer, Nanni Schmitt, Antje Knaflic, Verena Nowak, Nadine Weimer, Julia Oblaender, Iris Palme, Christiane Schumann, Claudia D. Baldus, Torsten J. Schulze, Patrick Wuchter, Henning Roehl, Ahmed Jawhar, Christel Weiss, Tobias Boch, Georgia Metzgeroth, Martin Neumann, Wolf-Karsten Hofmann, Florian Nolte, Daniel Nowak
Summary: The expression of ERFE and GDF15 in MDS patients was found to be highly aberrant, with ERFE expression unexpectedly relevant for MDS prognosis. ERFE overexpression predicted superior overall survival in certain MDS subgroups, suggesting a possible involvement of ERFE in the molecular pathogenesis of MDS.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Review
Medicine, General & Internal
Kathrin Nachtkamp, Guido Kobbe, Norbert Gattermann, Ulrich Germing
Summary: Myelodysplastic syndromes (MDS) are malignant diseases arising from hematopoietic stem cells, with an incidence of 4 cases per 100,000 persons per year. They are usually diagnosed when evaluating cytopenia, and the median survival time is three years. This review summarizes the latest research on the diagnosis and treatment of MDS.
DEUTSCHES ARZTEBLATT INTERNATIONAL
(2023)
Review
Immunology
Sarah Weber, Anastasia Parmon, Nina Kurrle, Frank Schnuetgen, Hubert Serve
Summary: MDS and AML patients often experience systemic iron overload issues, iron is closely associated with the pathogenesis and potential treatment strategies of the diseases, imbalances in iron homeostasis may lead to various cell death.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Oncology
Jessica M. Stempel, Nikolai A. Podoltsev, Talib Dosani
Summary: Myelodysplastic syndromes (MDS) are a group of bone marrow disorders characterized by ineffective hematopoiesis, progressive cytopenias, and the potential to progress to acute myeloid leukemia (AML). The most common causes of morbidity and mortality in MDS patients are complications related to MDS itself rather than progression to AML. Supportive care measures are essential, especially in lower-risk MDS patients with a better prognosis, who require long-term monitoring of disease and treatment-related complications. This review discusses the frequent complications and supportive care interventions in MDS patients, including transfusion support, iron overload management, antimicrobial prophylaxis, considerations in the era of COVID-19, routine immunizations, and palliative care.
Review
Medicine, General & Internal
Toni Valkovic, Marija Stanic Damic
Summary: Iron plays an important role in the survival and reproduction of fungal pathogens, and there is evidence linking invasive fungal infections to iron or iron overload. Understanding the association between iron and iron overload with susceptibility to invasive fungal infections is crucial, especially for patients with hematological malignancies.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Hematology
Maria Domenica Cappellini, Ali T. Taher, Amit Verma, Farrukh Shah, Olivier Hermine
Summary: Hematologic disorders like myelodysplastic syndromes and beta-thalassemia are characterized by ineffective erythropoiesis and anemia, usually managed through regular blood transfusions. However, treatments targeting the underlying mechanisms of ineffective erythropoiesis are limited, leading to a burden on patients, caregivers, and healthcare systems. This article provides an overview of the role of ineffective erythropoiesis in these disorders, discusses unmet needs in targeting it, and describes current management strategies and emerging treatments.
Article
Hematology
Ahmad Zargari, Simon Wu, Anthea Greenway, Ken Cheng, Zane Kaplan
Summary: In Australia, dual chelation therapy (DCT) has been found to effectively reduce serum ferritin levels and may contribute to improvement in liver iron content in patients with thalassaemia.
Article
Hematology
Jennifer C. Yui, Abdallah Geara, Farzana Sayani
Summary: This study presented cases of partial Fanconi syndrome associated with deferasirox in adults and suggested adjusting the dose of deferasirox based on ferritin levels and liver iron content. Monitoring with metabolic panel and urinalysis was found to be sufficient for detecting proximal tubular dysfunction associated with deferasirox.
Review
Medicine, General & Internal
Esther Natalie Oliva, Krystal Huey, Sohan Deshpande, Monica Turner, Madhura Chitnis, Emma Schiller, Derek Tang, Aylin Yucel, Christina Hughes, Farrukh Shah
Summary: High serum ferritin (SF) levels are associated with worse prognosis in MDS patients.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Hematology
Guillermo Garcia-Manero, Ghulam J. Mufti, Pierre Fenaux, Rena Buckstein, Valeria Santini, Maria Diez-Campelo, Carlo Finelli, Osman Ilhan, Mikkael A. Sekeres, Amer M. Zeidan, Rodrigo Ito, Jennie Zhang, Anita Rampersad, Daniel Sinsimer, Jay T. Backstrom, Uwe Platzbecker, Rami S. Komrokji
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
Orianne Wagner-Ballon, Peter Bettelheim, Jeroen Lauf, Frauke Bellos, Matteo Della Porta, Erica Travaglino, Dolores Subira, Irene Nuevo Lopez, Sihem Tarfi, Theresia M. Westers, Ulrika Johansson, Katherina Psarra, Serafeim Karathanos, Sergio Matarraz, Enrique Colado, Monali Gupta, Robin Ireland, Wolfgang Kern, Arjan A. Van de Loosdrecht
Summary: This multicenter study demonstrates the robustness of the monocyte assay in distinguishing chronic myelomonocytic leukemia from other causes of monocytosis. It also confirms the possibility of using this assay in bone marrow samples.
CYTOMETRY PART B-CLINICAL CYTOMETRY
(2023)
Article
Medical Laboratory Technology
Arjan A. Loosdrecht, Wolfgang Kern, Anna Porwit, Peter Valent, Sharham Kordasti, Eline Cremers, Canan Alhan, Carolien Duetz, Alan Dunlop, Willemijn Hobo, Frank Preijers, Orianne Wagner-Ballon, Nicolas Chapuis, Michaela Fontenay, Peter Bettelheim, Lisa Eidenschink-Brodersen, Patricia Font, Ulrika Johansson, Michael R. Loken, Jeroen G. Marvelde, Sergio Matarraz, Kiyoyuki Ogata, Uta Oelschlaegel, Alberto Orfao, Katherina Psarra, Dolores Subira, Denise A. Wells, Marie C. Bene, Matteo G. Della Porta, Kate Burbury, Frauke Bellos, Vincent H. J. van der Velden, Theresia M. Westers, Leonie Saft, Robin Ireland
Summary: This article discusses the rationale for including flow cytometry (FCM) in the diagnostic investigation and evaluation of cytopenias and suspected myelodysplastic syndromes (MDS) by the European LeukemiaNet international MDS Flow Working Group. The WHO 2016 classification acknowledges the contribution of FCM in the diagnosis of MDS and its potential usefulness in prognostication, prediction, therapy evaluation, and patient follow-up.
CYTOMETRY PART B-CLINICAL CYTOMETRY
(2023)
Article
Hematology
Carlo Visco, Luigi Marcheselli, Roberto Mina, Marianna Sassone, Anna Guidetti, Domenico Penna, Chiara Cattaneo, Valentina Bonuomo, Alessandro Busca, Andres Jose Maria Ferreri, Riccardo Bruna, Luigi Petrucci, Roberto Cairoli, Marco Salvini, Lorenza Bertu, Marco Ladetto, Sofia Pilerci, Antonello Pinto, Safaa Ramadan, Francesco Marchesi, Michele Cavo, Luca Arcaini, Elisa Coviello, Alessandra Romano, Pellegrino Musto, Massimo Massaia, Nicola Fracchiolla, Monia Marchetti, Annamaria Scattolin, Maria Chiara Tisi, Antonio Cuneo, Matteo Della Porta, Livio Trentin, Marco Turrini, Filippo Gherlinzoni, Agostino Tafuri, Sara Galimberti, Monica Bocchia, Valeria Cardinali, Daniela Cilloni, Alessandro Corso, Daniele Armiento, Luigi Rigacci, Elettra Ortu La Barbera, Carlo Gambacorti-Passerini, Giuseppe Visani, Daniele Vallisa, Adriano Venditti, Carmine Selleri, Annarita Conconi, Patrizia Tosi, Francesco Lanza, Anna Candoni, Mauro Krampera, Paolo Corradini, Francesco Passamonti, Francesco Merli
Summary: This study investigated the clinical behavior and clinical parameters of lymphoma patients with COVID-19, with the aim of identifying predictors of death. The results showed that lymphoma patients had poor outcomes when diagnosed with COVID-19, but treatment for lymphoma did not impact survival. The study also identified four easy-to-use factors associated with the risk of death.
Article
Hematology
Margherita Maffioli, Barbara Mora, Somedeb Ball, Alessandra Iurlo, Elena Maria Elli, Maria Chiara Finazzi, Nicola Polverelli, Elisa Rumi, Marianna Caramella, Maria Cristina Carraro, Mariella D'Adda, Alfredo Molteni, Cinzia Sissa, Francesca Lunghi, Alessandro Vismara, Marta Ubezio, Anna Guidetti, Sabrina Caberlon, Michela Anghilieri, Rami Komrokji, Daniele Cattaneo, Matteo Giovanni Della Porta, Toni Giorgino, Lorenza Bertu, Marco Brociner, Andrew Kuykendall, Francesco Passamonti
Summary: Ruxolitinib is commonly used in the treatment of myelofibrosis, but many patients lose response over time. This study identified predictors of survival and developed a prognostic model to identify patients who may benefit from a treatment switch.
Letter
Oncology
Rami S. Komrokji, Uwe Platzbecker, Pierre Fenaux, Amer M. Zeidan, Guillermo Garcia-Manero, Ghulam J. Mufti, Valeria Santini, Maria Diez-Campelo, Carlo Finelli, Joseph G. Jurcic, Peter L. Greenberg, Mikkael A. Sekeres, Amy E. DeZern, Michael R. Savona, Jeevan K. Shetty, Rodrigo Ito, George Zhang, Xianwei Ha, Jay T. Backstrom, Amit Verma
Letter
Oncology
Giulia Calabretto, Enrico Attardi, Antonella Teramo, Valentina Trimarco, Samuela Carraro, Sandra Mossuto, Gregorio Barila, Cristina Vicenzetto, Vanessa Rebecca Gasparini, Monica Crugnola, Pasquale Niscola, Antonella Poloni, Valentina Giai, Valentina Gaidano, Carlo Finelli, Roberta Bertorelle, Cinzia Candiotto, Marco Pizzi, Gianni Binotto, Monica Facco, Fabrizio Vianello, Livio Trentin, Gianpietro Semenzato, Renato Zambello, Valeria Santini
Review
Oncology
Federica Pilo, Daniela Cilloni, Matteo Giovanni Della Porta, Gian Luca Forni, Alberto Piperno, Valeria Santini, Emanuele Angelucci
Summary: This article reviews the recent understanding of the concept of exposure to free iron-mediated damage and emphasizes the importance of reducing organ exposure to ROS in order to prevent or delay organ damage.
Article
Medicine, General & Internal
Yao-Chung Liu, Junsu Kwon, Emiliano Fabiani, Zhijian Xiao, Yanjing V. Liu, Matilde Y. Follo, Jinqin Liu, Huijun Huang, Chong Gao, Jun Liu, Giulia Falconi, Lia Valentini, Carmelo Gurnari, Carlo Finelli, Lucio Cocco, Jin-Hwang Liu, Adrianna I. Jones, Junyu Yang, Henry Yang, Julie A. I. Thoms, Ashwin Unnikrishnan, John E. Pimanda, Rongqing Pan, Mahmoud A. Bassal, Maria T. Voso, Daniel G. Tenen, Li Chai
Summary: This study found that hypomethylating agents can induce demethylation and up-regulation of the oncogene SALL4 in certain cases, which is associated with poorer prognosis. The critical CpG island for SALL4 expression was identified in the 5' untranslated region. This discovery highlights the need for further investigation into the mechanisms of oncogene activation after treatment with hypomethylating agents.
NEW ENGLAND JOURNAL OF MEDICINE
(2022)
Review
Hematology
Carlo Finelli, Sarah Parisi, Stefania Paolini
Summary: Anemia is common in elderly patients with MDS and is associated with poor prognosis. The main objectives of RBC transfusion therapy are symptom control and improvement of HRQoL. However, transfusions can have negative clinical consequences, including adverse reactions and iron overload.
EXPERT REVIEW OF HEMATOLOGY
(2022)
Article
Oncology
Patrizia Zappasodi, Chiara Cattaneo, Virginia Valeria Ferretti, Roberto Mina, Andres Jose Maria Ferreri, Francesco Merli, Margherita Oberti, Mauro Krampera, Alessandra Romano, Caterina Zerbi, Jacqueline Ferrari, Michele Cavo, Marco Salvini, Lorenza Bertu, Nicola Stefano Fracchiolla, Francesco Marchesi, Massimo Massaia, Vincenzo Marasco, Roberto Cairoli, Anna Maria Scattolin, Alessandro Maria Vannucchi, Carlo Gambacorti-Passerini, Pellegrino Musto, Filippo Gherlinzoni, Antonio Cuneo, Antonello Pinto, Livio Trentin, Monica Bocchia, Sara Galimberti, Elisa Coviello, Maria Chiara Tisi, Alessandro Morotti, Brunangelo Falini, Mauro Turrini, Agostino Tafuri, Atto Billio, Massimo Gentile, Roberto Massimo Lemoli, Adriano Venditti, Matteo Giovanni Della Porta, Francesco Lanza, Luigi Rigacci, Patrizia Tosi, Sara Mohamed, Alessandro Corso, Mario Luppi, Nicola Giuliani, Alessandro Busca, Livio Pagano, Raffaele Bruno, Paolo Antonio Grossi, Paolo Corradini, Francesco Passamonti, Luca Arcaini
Summary: This study analyzed the impact of secondary infections in patients with hematological malignancies and COVID-19. It found that patients with secondary infections were older, had more comorbidities, and had worse outcomes from COVID-19.
HEMATOLOGICAL ONCOLOGY
(2022)
Article
Oncology
Barbara Mora, Paola Guglielmelli, Andrew Kuykendall, Elisa Rumi, Margherita Maffioli, Francesca Palandri, Valerio De Stefano, Marianna Caramella, Silvia Salmoiraghi, Jean-Jacques Kiladjian, Jason Gotlib, Alessandra Iurlo, Francisco Cervantes, Marco Ruggeri, Richard T. Silver, Francesco Albano, Giulia Benevolo, David M. Ross, Matteo G. Della Porta, Timothy Devos, Giada Rotunno, Rami S. Komrokji, Ilaria C. Casetti, Michele Merli, Marco Brociner, Domenica Caramazza, Giuseppe Auteri, Tiziano Barbui, Daniele Cattaneo, Lorenza Bertu, Luca Arcaini, Alessandro M. Vannucchi, Francesco Passamonti
Summary: Patients with Philadelphia-negative myeloproliferative neoplasms are at high risk of thrombotic events, and cytoreductive therapy can reduce the risk of thrombosis in patients with secondary myelofibrosis.
Review
Oncology
Vincenzo Guastafierro, Marta Ubezio, Nicla Manes, Chiara Milanesi, Matteo Della Porta, Arturo Bonometti
Summary: Chronic myelomonocytic leukemia (CMML) is a rare entity characterized by persistent peripheral blood monocytosis, hypercellular bone marrow, and dysplasia. This case report highlights a rare CSF3R-mutated CMML, which shares similarities with both CMML and CNL at clinical, pathological, and molecular levels. The diagnostic and therapeutic challenges posed by this unique mutation in CMML are emphasized.
LEUKEMIA & LYMPHOMA
(2023)
Meeting Abstract
Hematology
A. De Stefano, S. Mongiorgi, S. Ratti, M. Fogli, V. Salvestrini, A. Curti, M. Cavo, A. Pellagatti, J. Boultwood, L. Manzoli, L. Cocco, S. Parisi, S. Paolini, C. Finelli, M. Y. Follo