Article
Medicine, General & Internal
Huan Li, Fang Hu, Robert Peter Gale, Mikkael A. Sekeres, Yang Liang
Summary: Myelodysplastic syndromes (MDS) are a group of blood cancers characterized by dysregulated hematopoiesis and risk of transformation to acute myeloid leukemia. Prognostic systems can predict survival in MDS patients. Treatment goals differ for low-risk and high-risk MDS, with the aim to improve quality of life and prolong survival, respectively. Hematopoietic cell transplantation can cure MDS, but it is not widely used.
NATURE REVIEWS DISEASE PRIMERS
(2022)
Review
Oncology
Yan Jiang, Su-Jun Gao, Benoit Soubise, Nathalie Douet-Guilbert, Zi-Ling Liu, Marie-Berengere Troadec
Summary: Gene variants, particularly TP53 mutations, play a crucial role in the prognosis of myelodysplastic syndromes (MDS), being associated with higher risk categories, resistance to traditional therapies, rapid leukemic transformation, and poor outcomes. Current prognosis classification systems for MDS do not consider gene variants, but the impact is significant on the clinical heterogeneity and prognosis of the disease.
Review
Biochemistry & Molecular Biology
Ekaterina Balaian, Manja Wobus, Martin Bornhaeuser, Triantafyllos Chavakis, Katja Sockel
Summary: MDS is a clonal stem cell disorder characterized by genetic alterations, inflammatory processes, and changes in cellular metabolism. These abnormalities lead to dysfunction of HSPCs and niche cells, promoting clone expansion and increasing the risk for AML.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Medicine, General & Internal
Chloe Friedrich, Olivier Kosmider
Summary: This article discusses the role of the mesenchymal niche in the pathophysiology of Myelodysplastic syndromes (MDS) and provides an overview of currently available in vitro and in vivo models that can be used to study the effects of the mesenchymal niche on hematopoietic stem cells (HSCs).
Review
Oncology
Caner Saygin, Lucy A. Godley
Summary: Myelodysplastic syndrome (MDS) is a bone marrow disease characterized by the inability to produce normal numbers of blood cells. Different types of MDS are driven by distinct biological pathways, with advancements in treatment strategies based on molecular understanding. The recognition of precursor conditions may allow for strategies to delay or prevent MDS development.
Article
Oncology
Mikkael A. Sekeres, Nina Kim, Amy E. DeZern, Kelly J. Norsworthy, Jacqueline S. Garcia, R. Angelo de Claro, Marc R. Theoret, Emily Y. Jen, Lori A. Ehrlich, Amer M. Zeidan, Rami S. Komrokji
Summary: MDS drug development faces challenges in biology, trial design, and endpoints. To meet patient needs, approaches to improve MDS drug development should consider disease evolution, patient age, and real-world treatment conditions.
CLINICAL CANCER RESEARCH
(2023)
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)
Article
Oncology
Yasushige Aoyagi, Yoshihiro Hayashi, Yuka Harada, Kwangmin Choi, Natsumi Matsunuma, Daichi Sadato, Yuki Maemoto, Akihiro Ito, Shigeru Yanagi, Daniel T. Starczynowski, Hironori Harada
Summary: We demonstrated that excessive mitochondrial fragmentation is a fundamental pathobiological phenomenon that could trigger dysplasia formation and ineffective hematopoiesis in MDS. Our findings provide mechanistic insights into ineffective hematopoiesis and suggest dysregulated mitochondrial dynamics as a therapeutic target for treating MDS.
Article
Hematology
Krzysztof Madry, Karol Lis, Pierre Fenaux, David Bowen, Argiris Symeonidis, Moshe Mittelman, Reinhard Stauder, Jaroslav Cermak, Guillermo Sanz, Eva Hellstrom-Lindberg, Saskia Langemeijer, Luca Malcovati, Ulrich Germing, Mette Skov Holm, Agnes Guerci-Bresler, Dominic Culligan, Laurence Sanhes, Ioannis Kotsianidis, Corine van Marrewijk, Simon Crouch, Theo de Witte, Alex Smith
Summary: Information on causes of death and the impact of myelodysplastic syndromes (MDS) on survival in patients with lower-risk MDS is limited. This study shows that MDS and its related complications play a crucial role in the outcome of patients with lower-risk MDS.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Review
Oncology
Olga Kouroukli, Argiris Symeonidis, Periklis Foukas, Myrto-Kalliopi Maragkou, Eleni P. Kourea
Summary: This review examines the role of the bone marrow microenvironment in normal hematopoiesis, chronic inflammatory states, and myelodysplastic syndromes (MDS). It discusses the impact of cellular and biochemical immune parameters on the pathogenesis and evolution of MDS. The immune microenvironment of the bone marrow plays a critical role in both preventing and supporting the development of MDS, affecting normal hematopoiesis and the progression of the disease.
Review
Oncology
Sabine Blum, Gerasimos Tsilimidos, Helena Bresser, Michael Luebbert
Summary: Myelodysplasic syndromes (MDS) mainly occur in the elderly population, and most patients are too old or frail for intensive treatment. The current gold standard is treatment with hypomethylating agents, but the combination of hypomethylating agents with venetoclax has become the new gold standard for elderly and frail patients unable to undergo intensive treatment.
INTERNATIONAL JOURNAL OF CANCER
(2023)
Article
Multidisciplinary Sciences
Nanfang Huang, Yang Song, Wenhui Shi, Juan Guo, Lingyun Wu, Zheng Zhang, Qi He, Xiao Li, Feng Xu
Summary: DHX9 overexpression in myelodysplastic syndromes (MDS) is associated with poor prognosis and high risk of acute myeloid leukemia (AML) transformation. DHX9 is essential for malignant proliferation of leukemia cells. Suppression of DHX9 increases cell apoptosis and sensitizes cells to chemotherapy. Additionally, DHX9 knockdown inactivates PI3K-AKT and ATR-Chk1 signaling, promotes R-loop accumulation, and leads to R-loop-mediated DNA damage.
Review
Cell Biology
Mathieu Meunier, David Laurin, Sophie Park
Summary: The bone marrow niche plays an important role in the development of myelodysplastic syndromes. Mesenchymal stromal cells secrete extracellular vesicles and their miRNA, affecting the fate of hematopoietic stem cells and contributing to leukemogenesis. Extracellular vesicles containing miRNA and proteins can be used as diagnostic and prognostic markers for MDS. Targeting these vesicles or modulating their secretion may offer potential therapeutic directions for MDS.
Editorial Material
Hematology
Peter L. L. Greenberg
Summary: The paper by Zhang et al demonstrated the positive impact of combining the International Consensus Consortium (ICC) morphologic approach with prior clinical (IPSS-R) and mutational (IPSS-M) categorization to effectively subgroup and clinically evaluate MDS patients.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Review
Hematology
Nina Kim, Steven Pavletic, Kelly J. Norsworthy
Summary: The International Working Group (IWG) response criteria for myelodysplastic syndromes (MDS) have been widely adopted in clinical trials, but clinical experience has revealed limitations and the need for further validation of the endpoints proposed by the IWG.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Hematology
Silvia Ramos-Campoy, Anna Puiggros, Silvia Bea, Sandrine Bougeon, Maria Jose Larrayoz, Dolors Costa, Helen Parker, Gian Matteo Rigolin, Margarita Ortega, Maria Laura Blanco, Rosa Collado, Rocio Salgado, Tycho Baumann, Eva Gimeno, Carolina Moreno, Francesc Bosch, Xavier Calvo, Maria Jose Calasanz, Antonio Cuneo, Jonathan C. Strefford, Florence Nguyen-Khac, David Oscier, Claudia Haferlach, Jacqueline Schoumans, Blanca Espinet
Summary: This study compared the effectiveness of chromosome banding analysis (CBA) and genomic microarrays (GM) in risk stratification of CLL patients. The results showed moderate agreement between the two methods, but discordant classification in individual cases. Most discrepancies were technique-dependent and no significant correlation in the number of abnormalities was found when different filtering strategies were applied for GM. However, both CBA and GM showed similar predictive values for time to first treatment (TTFT) and overall survival. High complexity defined by both CBA and GM remained significant in the multivariate analysis for TTFT. Validation studies are needed to establish the prognostic value of genome complexity based on GM data in future prospective studies.
Article
Biophysics
Junya Kanda, Hiromi Hayashi, Annalisa Ruggeri, Fumihiko Kimura, Fernanda Volt, Satoshi Takahashi, Shinichi Kako, Karina Tozatto-Maio, Masamitsu Yanada, Guillermo Sanz, Naoyuki Uchida, Emanuele Angelucci, Seiko Kato, Mohamad Mohty, Edouard Forcade, Masatsugu Tanaka, Jorge Sierra, Takanori Ohta, Riccardo Saccardi, Takahiro Fukuda, Tatsuo Ichinohe, Takafumi Kimura, Vanderson Rocha, Shinichiro Okamoto, Arnon Nagler, Yoshiko Atsuta, Eliane Gluckman
Summary: The study evaluated the impact of GVHD on UCBT outcomes, finding that grade II acute GVHD had a positive impact on survival in the Japanese cohort but a negative impact in the European cohort. Grade III-IV acute GVHD had a negative impact on survival regardless of registries. Additionally, a positive impact of limited chronic GVHD on OS was observed only in the Japanese cohort.
BONE MARROW TRANSPLANTATION
(2022)
Letter
Oncology
Gail J. Roboz, Karen Yee, Amit Verma, Gautam Borthakur, Adolfo de la Fuente Burguera, Guillermo Sanz, Helai P. Mohammad, Ryan G. Kruger, Natalie O. Karpinich, Geraldine Ferron-Brady, Andre Acusta, Heather Del Buono, Therese Collingwood, Marc Ballas, Arindam Dhar, Andrew H. Wei
LEUKEMIA & LYMPHOMA
(2022)
Letter
Hematology
Maximilian Stahl, Omar Abdel-Wahab, Andrew H. Wei, Michael R. Savona, Mina L. Xu, Zhuoer Xie, Justin Taylor, Daniel Starczynowski, Guillermo F. Sanz, David A. Sallman, Valeria Santini, Gail J. Roboz, Mrinal M. Patnaik, Eric Padron, Olatoyosi Odenike, Aziz Nazha, Stephen D. Nimer, Ravindra Majeti, Richard F. Little, Steven Gore, Alan F. List, Vijay Kutchroo, Rami S. Komrokji, Tae Kon Kim, Nina Kim, Christopher S. Hourigan, Robert P. Hasserjian, Stephanie Halene, Elizabeth A. Griffiths, Peter L. Greenberg, Maria Figueroa, Pierre Fenaux, Fabio Efficace, Amy E. DeZern, Matteo G. Della Porta, Naval G. Daver, Jane E. Churpek, Hetty E. Carraway, Andrew M. Brunner, Uma Borate, John M. Bennett, Rafael Bejar, Jacqueline Boultwood, Sanam Loghavi, Jan Philipp Bewersdorf, Uwe Platzbecker, David P. Steensma, Mikkael A. Sekeres, Rena J. Buckstein, Amer M. Zeidan
Article
Medical Laboratory Technology
Rebeca Jurado, Maria Huguet, Blanca Xicoy, Marta Cabezon, Ari Jimenez-Ponce, David Quintela, Cristina De La Fuente, Minerva Raya, Esther Vinets, Jordi Junca, Joaquim Julia-Torras, Lurdes Zamora, Albert Oriol, Jose-Tomas Navarro, Xavier Calvo, Marc Sorigue
Summary: The choice of monocyte gating strategy has an impact on the quantification of monocyte subsets. Researchers designed a 10-color tube that allows proper monocyte gating even in highly abnormal peripheral blood. Computational analysis has the potential to increase the reproducibility of monocyte subset quantification.
CYTOMETRY PART B-CLINICAL CYTOMETRY
(2022)
Letter
Hematology
Marie Solange Flores-Moran, Juan Jose Rodriguez-Sevilla, Leonor Arenillas, Xavier Calvo, Ana Puiggros, Blanca Espinet, Beatriz Costan, Maria Rodriguez-Rivera, Marta Salido, Ana Ferrer del Alamo
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2023)
Letter
Oncology
Xavier Calvo
Article
Oncology
Maria Julia Montoro, Helena Pomares, Rosa Coll, Teresa Bernal del Castillo, Mar Tormo, Ana Jimenez, Salut Brunet, Javier Casano, Itziar Oiartzabal, Maria Diez-Campelo, Fernando Ramos, Rafael Romero, Eduardo Salido-Fierrez, Carmen Pedro, Joan Bargay, Carolina Munoz-Novas, Rocio Lopez, Montserrat Rafel, David Valcarcel
Summary: Myelodysplastic syndromes (MDS) is a heterogeneous group of diseases with variable treatment outcomes. This study assessed the disease progression of newly diagnosed and treatment-naive high-risk MDS patients and found that those who received active therapy early after diagnosis had better outcomes.
LEUKEMIA & LYMPHOMA
(2023)
Article
Oncology
Claudia Sargas, Rosa Ayala, Maria Jose Larrayoz, Maria Carmen Chillon, Estrella Carrillo-Cruz, Cristina Bilbao-Sieyro, Esther Prados de la Torre, David Martinez-Cuadron, Rebeca Rodriguez-Veiga, Blanca Boluda, Cristina Gil, Teresa Bernal, Juan Miguel Bergua, Lorenzo Algarra, Mar Tormo, Pilar Martinez-Sanchez, Elena Soria, Josefina Serrano, Juan Manuel Alonso-Dominguez, Raimundo Garcia-Boyero, Maria Luz Amigo, Pilar Herrera-Puente, Maria Jose Sayas, Esperanza Lavilla-Rubira, Joaquin Martinez-Lopez, Maria Jose Calasanz, Ramon Garcia-Sanz, Jose Antonio Perez-Simon, Maria Teresa Gomez-Casares, Joaquin Sanchez-Garcia, Eva Barragan, Pau Montesinos
Summary: Next-Generation Sequencing (NGS) has provided a deeper genetic understanding of acute myeloid leukemia (AML) and is becoming an irreplaceable diagnostic tool in clinical settings. The PETHEMA cooperative group has established a nationwide NGS network to study molecular profiles in AML patients, supporting the increasing role of NGS in clinical management.
Article
Oncology
Guadalupe Onate, Marta Pratcorona, Ana Garrido, Alicia Artigas-Baleri, Alex Bataller, Mar Tormo, Montserrat Arnan, Susana Vives, Rosa Coll, Olga Salamero, Ferran Vall-Llovera, Antonia Sampol, Antoni Garcia, Marta Cervera, Sara Garcia Avila, Joan Bargay, Xavier F. Ortin, Josep Nomdedeu, Jordi Esteve, Jorge Sierra
Summary: This study analyzed 227 patients with FLT3-mutated acute myeloid leukemia (AML) and found that the addition of midostaurin to treatment improved outcomes, especially in patients with NPM1 mutations.
BLOOD CANCER JOURNAL
(2023)
Article
Medicine, General & Internal
Pilar Solves, Javier Marco-Ayala, Miguel Angel Sanz, Ines Gomez-Segui, Aitana Balaguer-Rosello, Ana Facal, Marta Villalba, Juan Montoro, Guillermo Sanz, Javier de la Rubia, Jaime Sanz
Summary: This study compares the transfusion requirements of patients undergoing different modalities of hematopoietic stem cell transplantation (HSCT) over a twelve-year period. The results show that transfusion requirements have significantly increased for matched related donor HSCT, while there were no significant changes for matched unrelated donor and haploidentical transplant HSCT.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Hematology
Igor Stojkov, Annette Conrads-Frank, Ursula Rochau, Marjan Arvandi, Karin A. Koinig, Michael Schomaker, Moshe Mittelman, Pierre Fenaux, David Bowen, Guillermo F. Sanz, Luca Malcovati, Saskia Langemeijer, Ulrich Germing, Krzysztof Madry, Agnes Guerci-Bresler, Dominic J. Culligan, Ioannis Kotsianidis, Laurence Sanhes, Juliet Mills, Sibylle Puntscher, Daniela Schmid, Corine van Marrewijk, Alexandra Smith, Fabio Efficace, Theo de Witte, Reinhard Stauder, Uwe Siebert
Summary: This study aimed to identify determinants of low health-related quality of life (HRQoL) in patients recently diagnosed with myelodysplastic syndromes (MDS) for guiding early intervention strategies. Multiple factors, including age, sex, serum ferritin level, comorbidity burden, and Karnofsky performance status, influenced the HRQoL of MDS patients.
Letter
Hematology
Xavier Calvo, David Roman-Bravo, Nieves Garcia-Gisbert, Concepcion Fernandez-Rodriguez, Abora Rial-Villavecchia, Sara Garcia-Avila, Felix Lopez-Cadenas, Marta Salido, Ana Ferrer del alamo, Leonor Arenillas
INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY
(2023)
Letter
Oncology
Sergio Matarraz, Pilar Leoz, Ana Yeguas-Bermejo, Vincent van der Velden, Anne E. Bras, Jose I. Sanchez Gallego, Quentin Lecrevisse, Rosa Ayala-Bueno, Cristina Teodosio, Ignacio Criado, Maria Gonzalez-Gonzalez, Juan Flores-Montero, Alejandro Avendano, Maria B. Vidriales, Maria C. Chillon, Teresa Gonzalez, Ramon Garcia-Sanz, Maria I. Prieto Conde, Neus Villamor, Laura Magnano, Enrique Colado, Paula Fernandez, Edwin Sonneveld, Jan Philippe, Michaela Reiterova, Juan C. Caballero Berrocal, Francisco J. Diaz-Galvez, Fernando Ramos, Julio Davila Valls, Raquel Manjon Sanchez, Jackeline Solano Tovar, Xavier Calvo, Luis Garcia Alonso, Leonor Arenillas, Sara Alonso, Ariana Fonseca, Covadonga Quiros Caso, Jacques J. M. van Dongen, Alberto Orfao
BLOOD CANCER JOURNAL
(2023)
Article
Oncology
Sara Montesdeoca, Nieves Garcia-Gisbert, Xavier Calvo, Leonor Arenillas, David Roman, Concepcion Fernandez-Rodriguez, Rosa Navarro, Beatriz Costan, Maria del Carmen Vela, Laura Camacho, Eugenia Abella, Lluis Colomo, Marta Salido, Anna Puiggros, Lourdes Florensa, Blanca Espinet, Beatriz Bellosillo, Ana Ferrer del Alamo
Summary: This study found that Waldenstrom Macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with bone marrow involvement and IgM monoclonal gammopathy. The study further demonstrated the importance of peripheral blood (PB) involvement in the evaluation of patients with IgM monoclonal gammopathy and supported the role of PB multiparametric flow cytometry (MFC) analysis in the classification of mature B cell neoplasms.