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JOURNAL ARTICLE
Marta Isidro-Hernández, Ana Casado-García, Ninad Oak, Silvia Alemán-Arteaga, Belén Ruiz-Corzo, Jorge Martínez-Cano, Andrea Mayado, Elena G Sánchez, Oscar Blanco, Ma Luisa Gaspar, Alberto Orfao, Diego Alonso-López, Javier De Las Rivas, Susana Riesco, Pablo Prieto-Matos, África González-Murillo, Francisco Javier García Criado, María Begoña García Cenador, Manuel Ramírez-Orellana, Belén de Andrés, Carolina Vicente-Dueñas, César Cobaleda, Kim E Nichols, Isidro Sánchez-García
The initial steps of B-cell acute lymphoblastic leukemia (B-ALL) development usually pass unnoticed in children. Several preclinical studies have shown that exposure to immune stressors triggers the transformation of preleukemic B cells to full-blown B-ALL, but how this takes place is still a longstanding and unsolved challenge. Here we show that dysregulation of innate immunity plays a driving role in the clonal evolution of pre-malignant Pax5+/- B-cell precursors toward leukemia. Transcriptional profiling reveals that Myd88 is downregulated in immune-stressed pre-malignant B-cell precursors and in leukemic cells...
August 24, 2023: Nature Communications
#22
REVIEW
Peter Valent, Gregor Hoermann, Patrizia Bonadonna, Karin Hartmann, Wolfgang R Sperr, Sigurd Broesby-Olsen, Knut Brockow, Marek Niedoszytko, Olivier Hermine, Yannick Chantran, Joseph H Butterfield, Georg Greiner, Melody C Carter, Vito Sabato, Deepti H Radia, Frank Siebenhaar, Massimo Triggiani, Theo Gülen, Ivan Alvarez-Twose, Thomas Staudinger, Ludwig Traby, Karl Sotlar, Andreas Reiter, Hans-Peter Horny, Alberto Orfao, Stephen J Galli, Lawrence B Schwartz, Jonathan J Lyons, Jason Gotlib, Dean D Metcalfe, Michel Arock, Cem Akin
Physiologic levels of basal serum tryptase vary among healthy individuals, depending on the numbers of mast cells, basal secretion rate, copy numbers of the TPSAB1 gene encoding alpha tryptase, and renal function. Recently, there has been a growing debate about the normal range of tryptase because individuals with the hereditary alpha tryptasemia (HαT) trait may or may not be symptomatic, and if symptomatic, uncertainty exists as to whether this trait directly causes clinical phenotypes or aggravates certain conditions...
August 10, 2023: Journal of Allergy and Clinical Immunology in Practice
#23
JOURNAL ARTICLE
Cristina Bugarin, Laura Antolini, Chiara Buracchi, Sergio Matarraz, Tiziana Angela Coliva, Vincent H Van der Velden, Tomasz Szczepanski, Elaine Sobral Da Costa, Alita Van der Sluijs, Michaela Novakova, Ester Mejstrikova, Stefan Nierkens, Fabiana Vieira De Mello, Paula Fernandez, Carmen Aanei, Łukasz Sędek, Luisa Strocchio, Riccardo Masetti, Laura Sainati, Jan Philippé, Maria Grazia Valsecchi, Franco Locatelli, Jacques J M Van Dongen, Andrea Biondi, Alberto Orfao, Giuseppe Gaipa
Diagnostic criteria for juvenile myelomonocytic leukemia (JMML) are currently well defined, however in some patients diagnosis still remains a challenge. Flow cytometry is a well-established tool for diagnosis and follow-up of hematological malignancies, nevertheless it is not routinely used for JMML diagnosis. Herewith, we characterized the CD34+ hematopoietic precursor cells collected from 31 children with JMML using a combination of standardized EuroFlow antibody panels to assess the ability to discriminate JMML cells from normal/reactive bone marrow cell as controls (n=29) or from cells of children with other hematological diseases mimicking JMML (n=9)...
August 3, 2023: Haematologica
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JOURNAL ARTICLE
Nathalia Halax Orfão, Rubia Laine de Paula Andrade, Antônio Ruffino-Netto, Leila Wiedmann Florentino da Silva, Tereza Cristina Scatena Villa, Marva Lynn Seifert, Adriana Zilly, Marcos Augusto Moraes Arcoverde, Ismael Hoare, Ricardo Izurieta, Reinaldo Antônio da Silva-Sobrinho
BACKGROUND: To analyze the influence of the COVID-19 pandemic on the process of diagnosis and monitoring of drug-resistant pulmonary tuberculosis (TB) cases reported in the state of Paraná, Brazil, from 2015 to 2020. METHODS: Ecological study with quantitative approach. This study was based on diagnosed cases of pulmonary TB reported in the Notifiable Disease Information System in residents of Paraná; as well as through the number of confirmed cases of COVID-19 in the state epidemiological bulletin for the year 2020...
July 28, 2023: BMC Infectious Diseases
#25
JOURNAL ARTICLE
Joselina Antunes, João Órfão, João Rito, Cristina Adónis, Filipe Freire
PURPOSE: Investigate the effect of surgical treatment of obstructive sleep apnea syndrome (OSA) on sleep architecture. METHODS: Observational retrospective analysis of polysomnographic data of adults diagnosed with OSA, submitted to surgical treatment. Median (25-75th percentile) was used to present the data. RESULTS: Data were available for 76 adults, 55 men and 21 women, with median age of 49.0 years (41.0-62.0), body mass index of 27...
July 5, 2023: European Archives of Oto-rhino-laryngology
#26
JOURNAL ARTICLE
Ángela-Patricia Hernández, Laura Iglesias-Anciones, José Javier Vaquero-González, Rafael Piñol, Julio J Criado, Emilio Rodriguez, Pablo Juanes-Velasco, Marina L García-Vaquero, Carlota Arias-Hidalgo, Alberto Orfao, Ángel Millán, Manuel Fuentes
From chemistry design to clinical application, several approaches have been developed to overcome platinum drawbacks in antitumoral therapies. An in-depth understanding of intracellular signaling may hold the key to the relationship of both conventional drugs and nanoparticles. Within these strategies, first, nanotechnology has become an essential tool in oncotherapy, improving biopharmaceutical properties and providing new immunomodulatory profiles to conventional drugs mediated by activation of endoplasmic reticulum (ER) stress...
June 15, 2023: Cancers
#27
JOURNAL ARTICLE
Kyra van der Pan, Indu Khatri, Anniek L de Jager, Alesha Louis, Sara Kassem, Brigitta A E Naber, Inge F de Laat, Marjolijn Hameetman, Suzanne E T Comans, Alberto Orfao, Jacques J M van Dongen, Paula Díez, Cristina Teodosio
INTRODUCTION: Monitoring of innate myeloid cells (IMC) is broadly applied in basic and translational research, as well as in diagnostic patient care. Due to their immunophenotypic heterogeneity and biological plasticity, analysis of IMC populations typically requires large panels of markers. Currently, two cytometry-based techniques allow for the simultaneous detection of ≥40 markers: spectral flow cytometry (SFC) and mass cytometry (MC). However, little is known about the comparability of SFC and MC in studying IMC populations...
2023: Frontiers in Immunology
#28
JOURNAL ARTICLE
Mafalda Martins de Sousa, João Rebelo, Sónia Martins, Helena Silveira, Tiago Órfão, Carla Pinto Moura
INTRODUCTION: Septoplasty is one of the most common surgeries performed by otorhinolaryngologists. The gold standard for the evaluation of septal deviation is anterior rhinoscopy and nasal endoscopy. Frequently, computed tomography (CT) is also performed, although the correlation between septal deviation on CT and physical examination is unclear. OBJECTIVES: To study the relationship between symptoms and physical and radiological evaluation in patients who underwent septoplasty...
May 2023: Curēus
#29
JOURNAL ARTICLE
Natasha Z Anita, Felicia Kwan, Si Won Ryoo, Chelsi Major-Orfao, William Z Lin, Shiropa Noor, Krista L Lanctôt, Nathan Herrmann, Paul I Oh, Baiju R Shah, Jeremy Gilbert, Angela Assal, Ilana J Halperin, Ameer Y Taha, Walter Swardfager
Type 2 diabetes mellitus (T2DM) increases the risk of cognitive decline and dementia. Disruptions in the cytochrome P450-soluble epoxide hydrolase (CYP450-sEH) pathway have been reported in both T2DM and cognitive impairment. Obesity is common in this population and it has been associated with altered oxylipin concentrations. We examine linoleic acid (LA)-derived CYP450-sEH oxylipins and cognition in people with T2DM, and explore potential differences between obese and non-obese individuals. The study included 51 obese and 57 non-obese participants (mean age 63...
May 26, 2023: Journal of Lipid Research
#30
JOURNAL ARTICLE
Leire Burgos, Luis-Esteban Tamariz-Amador, Noemi Puig, Maria-Teresa Cedena, Camila Guerrero, Tomas Jelínek, Sarah Johnson, Paolo Milani, Lourdes Cordon, Jose J Perez, Marta Lasa, Rosalinda Termini, Albert Oriol, Miguel-Teodoro Hernandez, Luis Palomera, Rafael Martinez-Martinez, Javier de la Rubia, Felipe de Arriba, Rafael Rios, Maria-Esther Gonzalez, Mercedes Gironella, Valentin Cabañas, Maria Casanova, Isabel Krsnik, Albert Perez-Montaña, Verónica González-Calle, Paula Rodriguez-Otero, Vladimir Maisnar, Roman Hajek, Fritz Van Rhee, Victor Jimenez-Zepeda, Giovanni Palladini, Giampaolo Merlini, Alberto Orfao, Javier de la Cruz, Joaquin Martinez-Lopez, Juan-Jose Lahuerta, Laura Rosiñol, Joan Blade, Maria-Victoria Mateos, Jesus F San-Miguel, Bruno Paiva
PURPOSE: The existence of patients with multiple myeloma (MM) and light-chain (AL) amyloidosis who present with a monoclonal gammopathy of undetermined significance (MGUS)-like phenotype has been hypothesized, but methods to identify this subgroup are not standardized and its clinical significance is not properly validated. PATIENTS AND METHODS: An algorithm to identify patients having MGUS-like phenotype was developed on the basis of the percentages of total bone marrow (BM) plasma cells (PC) and of clonal PC within the BM PC compartment, determined at diagnosis using flow cytometry in 548 patients with MGUS and 2,011 patients with active MM...
June 1, 2023: Journal of Clinical Oncology
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JOURNAL ARTICLE
João Órfão, Marta Melo, Mafalda Trindade Soares, Diogo Raposo, Ana Rita Alemão, Leonel Barbosa, Filipe Freire
OBJECTIVE: To evaluate swallowing in patients who underwent CO2 LASER Partial Epiglottectomy (CO2-LPE) for Obstructive Sleep Apnea Syndrome and to assess the risk of aspiration with this technique. MATERIAL & METHODS: Chart review of adult patients who underwent CO2-LPE between 2016 and 2020, in a secondary care hospital. Patients underwent surgery for OSAS, in accordance to Drug Induced Sleep Endoscopy findings and an objective swallowing evaluation was done at least 6 months after surgery...
March 9, 2023: Auris, Nasus, Larynx
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JOURNAL ARTICLE
Anna Beatriz Dos Santos Salgado, Roberto Jose Pessoa Magalhães, Robéria M Pontes, Eduarda da Silva Barbosa, Juan Flores-Montero, Luzalba Sanoja-Flores, Marcelo Gerardin Poirot Land, Glicinia Pimenta, Hélio Dos Santos Dutra, Elaine S Costa, Alberto Orfao, Angelo Maiolino
Despite recent advances in multiple myeloma (MM), the incorporation of novel agents and measurable residual disease (MRD) monitoring in low-income countries remains a challenge. Although lenalidomide maintenance (M-Len) after autologous stem cell transplantation (ASCT) has been associated with improved outcomes and MRD has refined the prognosis of complete response (CR) cases, until now, there have been no data on the benefits of these approaches in Latin America. Here, we evaluate the benefits of M-Len and MRD using next-generation flow cytometry (NGF-MRD) at Day + 100 post-ASCT ( n = 53)...
March 4, 2023: Cancers
#33
REVIEW
Peter Valent, Karin Hartmann, Patrizia Bonadonna, Wolfgang R Sperr, Marek Niedoszytko, Olivier Hermine, Hanneke C Kluin-Nelemans, Karl Sotlar, Gregor Hoermann, Boguslaw Nedoszytko, Sigurd Broesby-Olsen, Roberta Zanotti, Magdalena Lange, Michael Doubek, Knut Brockow, Ivan Alvarez-Twose, Judit Varkonyi, Selim Yavuz, Gunnar Nilsson, Deepti Radia, Clive Grattan, Juliana Schwaab, Theo Gülen, Hanneke N G Oude Elberink, Hans Hägglund, Frank Siebenhaar, Emir Hadzijusufovic, Vito Sabato, Jiri Mayer, Andreas Reiter, Alberto Orfao, Hans-Peter Horny, Massimo Triggiani, Michel Arock
In 2002, the European Competence Network on Mastocytosis (ECNM) was launched as a multidisciplinary collaborative initiative to increase the awareness and to improve diagnosis and management of patients with mast cell (MC) disorders. The ECNM consists of a net of specialized centers, expert physicians, and scientists who dedicate their work to MC diseases. One essential aim of the ECNM is to timely distribute all available information about the disease to patients, doctors, and scientists. In the past 20 years, the ECNM has expanded substantially and contributed successfully to the development of new diagnostic concepts, and to the classification, prognostication, and treatments of patients with mastocytosis and MC activation disorders...
March 1, 2023: Journal of Allergy and Clinical Immunology in Practice
#34
JOURNAL ARTICLE
Livio Pagano, Pier Luigi Zinzani, Stefano Pileri, Pietro Quaglino, Branko Cuglievan, Emilio Berti, Naveen Pemmaraju, Francesco Onida, Rein Willemze, Alberto Orfao, Giovanni Barosi
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a hematological malignancy characterized by recurrent skin nodules, an aggressive clinical course with rapid involvement of hematological organs, and a poor prognosis with overall survival. The rarity of the disease results in a few large-scale studies, a lack of controlled clinical trials for its management, and a lack of evidence-based guidelines. Here, we present a review of unmet clinical needs on the management of BPDCN by a panel of eleven experts involved in the research and clinical practice of BPDCN...
March 2023: HemaSphere
#35
JOURNAL ARTICLE
Tiago Azenha Rama, Ana Filipa Henriques, Almudena Matito, Maria Jara-Acevedo, Carolina Caldas, Andrea Mayado, Javier I Muñoz-González, André Moreira, João Cavaleiro-Rufo, Andrés García-Montero, Alberto Órfão, Laura Sanchez-Muñoz, Iván Álvarez-Twose
BACKGROUND: Mastocytosis encompasses a heterogeneous group of diseases characterized by tissue accumulation of clonal mast cells, which frequently includes bone involvement. Several cytokines have been shown to play a role in the pathogenesis of bone mass loss in systemic mastocytosis (SM), but their role in SM-associated osteosclerosis remains unknown. OBJECTIVE: To investigate the potential association between cytokine and bone remodeling markers with bone disease in SM, aiming at identifying biomarker profiles associated with bone loss and/or osteosclerosis...
May 2023: Journal of Allergy and Clinical Immunology in Practice
#36
JOURNAL ARTICLE
Alicia Landeira-Viñuela, Miguel Alcoceba-Sanchez, Almudena Navarro-Bailón, Carlota Arias-Hidalgo, Pablo Juanes-Velasco, José Manuel Sánchez-Santos, Quentin Lecrevisse, Carlos Eduardo Pedreira, Marina L García-Vaquero, Ángela-Patricia Hernández, Enrique Montalvillo, Rafael Góngora, Javier De Las Rivas, Marcos González-Díaz, Alberto Orfao, Manuel Fuentes
UNLABELLED: Chronic lymphocytic leukemia (CLL) is the most common leukemia in the Western world. Studies of CLL antibody reactivity have shown differential targets to autoantigens and antimicrobial molecular motifs that support the current hypothesis of CLL pathogenesis. METHODS: In this study, we conducted a quantitative serum analysis of 7 immunoglobulins in CLL and monoclonal B-cell lymphocytosis (MBL) patients (bead-suspension protein arrays) and a serological profile (IgG and IgM) study of autoantibodies and antimicrobial antigens (protein microarrays)...
January 31, 2023: Cancers
#37
JOURNAL ARTICLE
Inês Órfão, Constança Carvalho, Inês Rodrigues, Leonor Ascensão, Marie Pedaccini, Luís Vicente, Miguel Barbosa, Susana A M Varela
BACKGROUND: Evidence of male-male courtship display is widespread across the animal kingdom. Yet, its function and evolutionary origin remain unclear. Here, we hypothesise that male-male courtship display evolved in response to selection pressure exerted by intrasexual competition during male-female courtship interactions. Intrasexual competition can be caused by bystander male pressure through eavesdropping and exploiting on displayer male's courtship interactions with females. This bystander pressure can lead to an audience effect by the displayer, who will change their courtship behaviour in the presence of bystanders and display directly towards them, even in the absence of females, as an intimidation strategy...
2023: PeerJ
#38
REVIEW
Helena Miriam Lorenzo Juanes, Cristina Carbonell, Begoña Febrer Sendra, Amparo López-Bernus, Alberto Bahamonde, Alberto Orfao, Carmen Vieira Lista, María Sánchez Ledesma, Ana Isabel Negredo, Beatriz Rodríguez-Alonso, Beatriz Rey Bua, María Paz Sánchez-Seco, Juan Luis Muñoz Bellido, Antonio Muro, Moncef Belhassen-García
Crimean-Congo hemorrhagic fever (CCHF) is a viral infectious disease for which distribution of the main vector, Hyalomma spp. ticks, is expanding. We analyzed all 10 cases of CCHF diagnosed in Spain during 2013-2021; case-patient median age was 56.5 years, and 7 were men. We identified CCHF virus genotypes III and V. Six case-patients acquired the infection in urban areas. Sixty percent of patients were infected in summer and 40% in spring. Two patients met criteria for hemophagocytic syndrome. Seven patients survived...
February 2023: Emerging Infectious Diseases
#39
JOURNAL ARTICLE
Patricia Alejandra Garrido Ruiz, María González-Tablas, Alejandro Pasco Peña, María Victoria Zelaya Huerta, Javier Ortiz, Álvaro Otero, Luis Antonio Corchete, María Dolores Ludeña, María Cristina Caballero Martínez, Alicia Córdoba Iturriagagoitia, Inmaculada Catalina Fernández, Joaquín González-Carreró Fojón, Aurelio Hernández Laín, Alberto Orfao, María Dolores Tabernero
Rhabdoid meningiomas (RM) shows heterogeneous histological findings, and a wide variety of chromosomal copy number alterations (CNA) are associated with an unpredictable course of the disease. In this study, we analyzed a series of 305 RM samples from patients previously reported in the literature and 33 samples from 23 patients studied in our laboratory. Monosomy 22-involving the minimal but most common recurrent region loss of the 22q11.23 chromosomal region was the most observed chromosomal alteration, followed by losses of chromosomes 14, 1, 6, and 19, polysomies of chromosomes 17, 1q, and 20, and gains of 13q14...
January 6, 2023: International Journal of Molecular Sciences
#40
JOURNAL ARTICLE
Ignacio Criado, Wendy G Nieto, Guillermo Oliva-Ariza, Blanca Fuentes-Herrero, Cristina Teodosio, Quentin Lecrevisse, Antonio Lopez, Alfonso Romero, Julia Almeida, Alberto Orfao, The Primary Health Care Group Of Salamanca For The Study Of Mbl
Reference ranges of blood-circulating leukocyte populations by, e.g., age and sex, are required for monitoring immune-cell kinetics. Most previous reports in which flow cytometry has been used to define the reference ranges for leukocyte counts included a limited number of donors and/or cell populations and/or did not consider age and sex simultaneously. Moreover, other factors not previously considered in the definition of normal ranges, such as the presence of chronic-lymphocytic-leukemia (CLL)-like low-count monoclonal B-cell lymphocytosis (MBLlo ), might also be associated with an altered distribution of leukocytes in blood in association with an immunodeficiency and increased risk of infection and cancer...
December 22, 2022: Cancers
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