Review
Oncology
Marie Donzel, Lucile Baseggio, Juliette Fontaine, Florian Pesce, Herve Ghesquieres, Emmanuel Bachy, Aurelie Verney, Alexandra Traverse-Glehen
Summary: Splenic marginal zone lymphoma (SMZL) is a small B-cell lymphoma with mostly indolent progression, but a third of patients may rapidly progress and require aggressive treatments. Recent advances in molecular biology techniques have led to new discoveries in understanding SMZL physiopathology, highlighting the need for updating current knowledge to guide future research.
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
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
Oncology
Juan Jose Rodriguez-Sevilla, Antonio Salar
Summary: MALT lymphoma is the most common subtype of marginal zone lymphomas, characterized by indolent behavior and diverse genetic alterations. Chromosomal translocations and mutations in several genes play key roles in the development of MALT lymphoma. Advances in next-generation sequencing technologies have provided insights into the mutational landscape and potential therapeutic targets. This comprehensive review summarizes the genetic landscape of MALT lymphomas and highlights the potential of precision medicine in the treatment of this disease.
Article
Oncology
William J. Archibald, Andrea M. Baran, AnnaLynn M. Williams, Rabih M. Salloum, W. Richard Burack, Andrew G. Evans, Chauncey R. Syposs, Clive S. Zent
Summary: This study investigated the diagnostic and therapeutic role of splenectomy for non-cHCL indolent splenic B-cell lymphomas. Splenectomy can be considered as a diagnostic and therapeutic option for patients with suspected non-cHCL splenic lymphomas.
Article
Hematology
Baptiste Gailllard, Pascale Cornillet-Lefebvre, Quoc-Hung Le, Karim Maloum, Melanie Pannetier, Carinne Lecoq-Lafon, Beatrice Grange, Ludovic Jondreville, Lucienne Michaux, Nathalie Nadal, Antoine Ittel, Isabelle Luquet, Stephanie Struski, Christine Lefebvre, Jean-Baptiste Gaillard, Marina Lafage-Pochitaloff, Estelle Balducci, Dominique Penther, Carole Barin, Marie Agnes Collonge-Rame, Melanie Jimenez-Poquet, Steven Richebourg, Pierre Lemaire, Sabine Defasque, Isabelle Radford-Weiss, Audrey Bidet, Santos A. Susin, Florence Nguyen-Khac, Elise Chapiro
Summary: Translocations involving the CDK6 gene are a rare but recurrent abnormality in B-cell neoplasms, mainly manifesting as chronic lymphocytic leukaemia, monoclonal B-cell lymphocytosis, or small lymphocytic lymphoma. The most common subtype affected is marginal zone lymphoma, with the majority of cases showing distinctive features.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
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
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)
Article
Immunology
Artur Kibler, Bettina Budeus, Ralf Kueppers, Marc Seifert
Summary: The composition of human splenic marginal zone (sMZ) B cells changes significantly with age, with a major population of lowly Ig-mutated CD27neg but antigen-experienced B cells early in life.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Pathology
James R. Cook, Catalina Amador, Magdalena Czader, Amy Duffield, John Goodlad, German Ott, Wenbin Xiao, Sandeep Dave, Devang Thakkar, Elizabeth Thacker, Ahmet Dogan, Mariusz Wasik, Reza Nejati
Summary: The conclusions of the 2021 Society for Hematopathology/European Association for Haematopathology workshop on transformations of marginal zone lymphoma (MZL) and lymphoplasmacytic lymphoma (LPL) were summarized. The most common transformation is to diffuse large B-cell lymphoma (DLBCL), but there are also other transformation events to different types of lymphomas. Comprehensive analysis, including sequencing studies, can aid in the diagnosis and classification of these rare cases.
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
(2023)
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.
Review
Oncology
Karima Amaador, Catherine Thieblemont, Judith Trotman, Monique C. Minnema
Summary: Marginal Zone Lymphoma (MZL) and Waldenstrom's Macroglobulinemia (WM) are indolent lymphomas that can be differentiated by the MYD88 L256P somatic mutation. Both MZL and WM patients have new treatment options available due to several large clinical studies with new drugs. This short review discusses the recent literature and provides recommendations.
HEMATOLOGICAL ONCOLOGY
(2023)
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
Oncology
Patricia Johansson, Anja Eckstein, Ralf Kueppers
Summary: Ocular adnexal marginal zone lymphoma (OAMZL) is a distinct type of lymphoma that occurs in tissues around the eyeball. The development of this lymphoma is associated with the constitutive activation of the NF-kappa B pathway, which may be caused by genetic alterations and epigenetic changes. The mucosa-associated lymphatic tissue (MALT) subtype is the most common subtype of lymphoma in the ocular adnexa.
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)
Article
Hematology
Antonio Sacco, Vanessa Desantis, Jon Celay, Viviana Giustini, Fabio Rigali, Francesco D. Savino, Michele Cea, Debora Soncini, Antonia Cagnetta, Antonio G. Solimando, Deborah D'Aliberti, Silvia Spinelli, Daniele Ramazzotti, Camillo Almici, Katia Todoerti, Antonino Neri, Antonella Anastasia, Alessandra Tucci, Marina Motta, Marco Chiarini, Yawara Kawano, Jose A. Martinez-Climent, Rocco Piazza, Aldo M. Roccaro
Summary: Recent investigations have shown that Waldenstrom macroglobulinemia (WM) exhibits an increased number of regulatory T cells (Tregs), and Tregs derived from patients with WM have a peculiar mRNA signature and functional phenotype. WM cells trigger significantly higher induction, expansion, and proliferation of Tregs compared to normal cells, especially in the context of CXCR4(C1013G)-mutated WM cells. CD40/CD40-ligand interaction is identified as an important axis supporting the interaction between WM cells and Tregs.
Meeting Abstract
Hematology
Jesus Maria Hernandez-Rivas, Inmaculada Serramito-Gomez, Saara Kallio, Amparo Cantalejo, Jana Kotaskova, Ana E. Rodriguez, Maria Abaigar, Rocio Benito, Anna Dolnik, Mark Catherwood, Jaine Blayney, Boris Tichy, Ivana Chalupova, Philippe Dubois, Roberto Lopez, Carlos Barranquero, Maria Diaz Lorenzo, Mylene Leitzelman, Sadiksha Adhikari, Sarka Pospisilova, Lars Bullinger, Ken Mills, Caroline A. Heckman
Article
Hematology
Ana Marin-Quilez, Christian Andrea Buduo, Lorena Diaz-Ajenjo, Vittorio Abbonante, Elena Vuelta, Paolo Maria Soprano, Cristina Miguel-Garcia, Sandra Santos-Minguez, Inmaculada Serramito-Gomez, Pedro Ruiz-Sala, Maria Jesus Penarrubia, Emilia Pardal, Jesus Maria Hernandez-Rivas, Jose Ramon Gonzalez-Porras, Ignacio Garcia-Tunon, Rocio Benito, Jose Rivera, Alessandra Balduini, Jose Maria Bastida
Summary: In this study, 4 variants affecting GALE were identified in 3 patients with severe thrombocytopenia. These patients exhibited giant and/or grey platelets, impaired platelet aggregation, and reduced secretion of alpha and dense granules. The study also revealed the critical role of GALE in the glycosylation and externalization of key proteins involved in platelet production and function, such as GPIb alpha-IX-V and mature beta 1 integrin.
Article
Biochemistry & Molecular Biology
Elisa Felix-Soriano, Neira Sainz, Marta Fernandez-Galilea, Eva Gil-Iturbe, Jon Celay, Jose A. Martinez-Climent, Maria J. Moreno-Aliaga
Summary: This study aimed to investigate the potential beneficial effects of chronic docosahexaenoic acid (DHA) supplementation on restoring subcutaneous white adipose tissue (scWAT) plasticity in obese aged female mice. The results showed that the DHA-enriched diet reduced adipocyte size and reversed the upregulation of lipogenic genes induced by the high fat diet (HFD). DHA supplementation restored the increase of proinflammatory genes, switched scWAT macrophages profile to a healthier phenotype, and induced beige adipocyte markers in obese aged mice.
JOURNAL OF NUTRITIONAL BIOCHEMISTRY
(2023)
Correction
Oncology
Larry Mansouri, Birna Thorvaldsdottir, Lesley-Ann Sutton, Georgios Karakatsoulis, Manja Meggendorfer, Helen Parker, Ferran Nadeu, Christian Brieghel, Stamatia Laidou, Riccardo Moia, Davide Rossi, Mark Catherwood, Jana Kotaskova, Julio Delgado, Ana E. Rodriguez-Vicente, Rocio Benito, Gian Matteo Rigolin, Silvia Bonfiglio, Lydia Scarfo, Mattias Mattsson, Zadie Davis, Ajay Gogia, Lata Rani, Panagiotis Baliakas, Hassan Foroughi-Asl, Cecilia Jylha, Aron Skaftason, Inmaculada Rapado, Fatima Miras, Joaquin Martinez-Lopez, Javier de la Serna, Jesus Maria Hernandez Rivas, Patrick Thornton, Maria Jose Larrayoz, Maria Jose Calasanz, Viktoria Fesus, Zoltan Matrai, Csaba Bodor, Karin E. Smedby, Blanca Espinet, Anna Puiggros, Ritu Gupta, Lars Bullinger, Francesc Bosch, Barbara Tazon-Vega, Fanny Baran-Marszak, David Oscier, Florence Nguyen-Khac, Thorsten Zenz, Maria Jose Terol, Antonio Cuneo, Maria Hernandez-Sanchez, Sarka Pospisilova, Ken Mills, Gianluca Gaidano, Carsten U. Niemann, Elias Campo, Jonathan C. Strefford, Paolo Ghia, Kostas Stamatopoulos, Richard Rosenquist
Review
Biochemistry & Molecular Biology
Ana Marin-Quilez, Lorena Diaz-Ajenjo, Christian A. A. Di Buduo, Ana Zamora-Canovas, Maria Luisa Lozano, Rocio Benito, Jose Ramon Gonzalez-Porras, Alessandra Balduini, Jose Rivera, Jose Maria Bastida
Summary: Protein glycosylation, including sialylation, is important for various biological processes. Carbohydrate conjugation to molecules and receptors is critical for normal hematopoiesis and platelet regulation. More than 200 enzymes are involved in proper glycosylation and sialylation. Genetic alterations in GNE, SLC35A1, GALE, and B4GALT can lead to syndromic manifestations, severe inherited thrombocytopenia, and hemorrhagic complications.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Cell Biology
Ana Marin-Quilez, Christian A. Di Buduo, Rocio Benito, Alessandra Balduini, Jose Rivera, Jose Maria Bastida
Summary: The GALE gene encodes an enzyme called UDP-galactose-4-epimerase, which plays a crucial role in the biosynthesis of glycoproteins and glycolipids. GALE-related disorders, such as galactosemia, are inherited in an autosomal recessive pattern and can lead to various complications. Recent studies have also linked GALE variants to severe thrombocytopenia and myelodysplastic syndrome.
Article
Oncology
Borja Puertas, Veronica Gonzalez-Calle, Eduardo Sobejano-Fuertes, Fernando Escalante, Jose A. Queizan, Abelardo Barez, Jorge Labrador, Jose Maria Alonso-Alonso, Alfonso Garcia de Coca, Alberto Cantalapiedra, Teresa Villaescusa, Carlos Aguilar-Franco, Elena Alejo-Alonso, Beatriz Rey-Bua, Lucia Lopez-Corral, Ramon Garcia-Sanz, Noemi Puig, Norma C. Gutierrez, Maria-Victoria Mateos
Summary: Survival of multiple myeloma patients has improved over time with the development of new treatments, especially with the combination of novel agents. Using at least two novel agents in induction therapy significantly prolongs overall survival, even in older patients. This suggests that multiple myeloma has become a chronic and potentially curable disease in a subset of patients with the current therapeutic approaches.
Article
Biochemistry & Molecular Biology
Juan Carlos Caballero, Elham Askari, Nerea Carrasco, Miguel Angel Piris, Begona Perez de Camino, Laura Pardo, Javier Cornago, Jose Luis Lopez-Lorenzo, Pilar Llamas, Laura Solan
Summary: This article reports a case of a 77-year-old woman diagnosed with Waldenstrom macroglobulinemia (WM) following severe and sudden pancytopenia associated with cold agglutinin syndrome. Treatment with rituximab, corticosteroids, and cyclophosphamide was initiated to manage WM and underlying hemolysis. Despite improvements in hemolysis parameters, pancytopenia persisted and ibrutinib was started as a second-line treatment. The patient developed an uncommon invasive fungal infection (IFI) with bone marrow granulomatosis and myelofibrosis during treatment, highlighting a poor hematopoietic response and numerous complications.
Article
Medicine, General & Internal
Juan Carlos Caballero, Laura Pardo, Maria Socorro Rodriguez-Pinilla, Miguel Angel Piris, Beatriz Alvarez, Laura Solan, Javier Cornago, Jose Luis Lopez-Lorenzo, Pilar Llamas, Raul Cordoba, Alberto Lopez-Garcia
Summary: This article presents a rare case of a 25-year-old patient diagnosed with human immunodeficiency virus (HIV) and the development of primary effusion lymphoma (PEL), Kaposi's sarcoma (KS), and multicentric Castleman's disease (MCD). Despite intensive treatment, the patient's condition did not improve. This case highlights the need for new therapies and research in this field.
MEDICINA-LITHUANIA
(2023)
Article
Hematology
Irene Peris, Silvia Romero-Murillo, Elena Martinez-Balsalobre, Caroline C. Farrington, Elena Arriazu, Nerea Marcotegui, Marta Jimenez-Munoz, Cristina Alburquerque-Prieto, Andrea Torres-Lopez, Vicente Fresquet, Jose A. Martinez-Climent, Maria C. Mateos, Maria L. Cayuela, Goutham Narla, Maria D. Odero, Carmen Vicente
Summary: Venetoclax combination therapies are becoming the standard of care in AML, but their efficacy in older/unfit patients is limited, highlighting the need for more effective therapies. Reactivating protein phosphatase 2A (PP2A) enhances venetoclax activity in AML cells by modulating BCL2 dependency and extracellular signal-regulated kinase signaling. Targeting PP2A increases the efficacy of venetoclax and azacitidine combination therapy in AML.
Meeting Abstract
Oncology
Beatriz Rey-Bua, Sofi A. Toribio-Castello, Merelyn Bonome-Gonzalez, Angela Villaverde-Ramiro, Teresa Gonzalez, Monica Del Rey, Jesus Maria Hernandez-Rivas, Ana Yeguas, Alejandro Avendano, Sandra Patricia Gomez-Ubeda, Marta Fonseca Santos, Borja Puertas, Elena Alejo Alonso, Maria Diez-Campelo
CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
(2022)
Meeting Abstract
Oncology
Jordi Ribera, Isabel Granada, Teresa Gonzalez, Mireia Morgades, Ricardo Sanchez, Esperanza Such, Susana Barrena, Juana Ciudad, Beatriz Soriano, Rocio Benito, Gayane Avetisyan, Eva Lumbreras, Cristina Miguel, Sandra Santos, Lurdes Zamora, Mar Mallo, Eulalia Genesca, Celia Gonzalez, Thaysa Lopes, Jesus-Maria Hernandez-Rivas, Alberto Orfao, Josep Maria Ribera
CLINICAL LYMPHOMA MYELOMA & LEUKEMIA
(2022)
