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1. Serum chemistry profiling and prognostication in systemic mastocytosis: a registry-based study of the ECNM and GREM

Author

Lübke, Johannes, Schmid, Alicia, Christen, Deborah, Oude Elberink, Hanneke N. G., Span, Lambert F. R., Niedoszytko, Marek, Gorska, Aleksandra, Lange, Magdalena, Gleixner, Karoline V., Hadzijusufovic, Emir, Stefan, Alex, Angelova-Fischer, Irena, Zanotti, Roberta, Bonifacio, Massimiliano, Bonadonna, Patrizia, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Müller, Sabine, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Hagglund, Hans, Mattsson, Mattias, Parente, Roberta, Varkonyi, Judit, Fortina, Anna Belloni, Caroppo, Francesca, Brockow, Knut, Zink, Alexander, Breynaert, Christine, Leven, Toon, Yavuz, Akif Selim, Doubek, Michael, Sabato, Vito, Schug, Tanja, Hartmann, Karin, Triggiani, Massimo, Gotlib, Jason, Hermine, Olivier, Arock, Michel, Kluin-Nelemans, Hanneke C., Panse, Jens, Sperr, Wolfgang R., Valent, Peter, Reiter, Andreas, and Schwaab, Juliana

Published
2024
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2. Severe Asthma Network Italy Definition of Clinical Remission in Severe Asthma: A Delphi Consensus

Author

Canonica, Giorgio Walter, Blasi, Francesco, Carpagnano, Giovanna Elisiana, Guida, Giuseppe, Heffler, Enrico, Paggiaro, Pierluigi, Allegrini, Chiara, Antonelli, Andrea, Aruanno, Arianna, Bacci, Elena, Bagnasco, Diego, Beghè, Bianca, Bonavia, Marco, Bonini, Matteo, Brussino, Luisa, Caiaffa, Maria Filomena, Calabrese, Cecilia, Camiciottoli, Gianna, Caminati, Marco, Caruso, Cristiano, Cavallini, Mirta, Chieco Bianchi, Fulvia, Conte, Maria Elisabetta, Corsico, Angelo Guido, Cosmi, Lorenzo, Costantino, Mariateresa, Costanzo, Giulia, Crivellaro, Mariaangiola, D'Alò, Simona, D'Amato, Mariella, Detoraki, Aikaterini, Di Proietto, Maria Carmela, Facciolongo, Nicola Cosimo, Ferri, Sebastian, Fierro, Vincenzo, Foschino, Maria Pia, Latorre, Manuela, Lombardi, Carlo, Macchia, Luigi, Milanese, Manlio, Montagni, Marcello, Parazzini, Elena Maria, Parente, Roberta, Passalacqua, Giovanni, Patella, Vincenzo, Pelaia, Girolamo, Pini, Laura, Puggioni, Francesca, Ricciardi, Luisa, Ridolo, Erminia, Rolo, Joyce, Scichilone, Nicola, Scioscia, Giulia, Senna, Gianenrico, Solidoro, Paolo, Varricchi, Gilda, Vianello, Andrea, Yacoub, Mona Rita, and Yang, Baoran

Published
2023
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5. Prognostic Impact of Organomegaly in Mastocytosis: An Analysis of the European Competence Network on Mastocytosis

Author

Lübke, Johannes, Schwaab, Juliana, Christen, Deborah, Elberink, Hanneke Oude, Span, Bart, Niedoszytko, Marek, Gorska, Aleksandra, Lange, Magdalena, Gleixner, Karoline V., Hadzijusufovic, Emir, Solomianyi, Oleksii, Angelova-Fischer, Irena, Zanotti, Roberta, Bonifacio, Massimiliano, Bonadonna, Patrizia, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Müller, Sabine, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Hagglund, Hans, Mattsson, Mattias, Parente, Roberta, Varkonyi, Judit, Fortina, Anna Belloni, Caroppo, Francesca, Zink, Alexander, Brockow, Knut, Breynaert, Christine, Bullens, Dominique, Yavuz, Akif Selim, Doubek, Michael, Sabato, Vito, Schug, Tanja, Niederwieser, Dietger, Hartmann, Karin, Triggiani, Massimo, Gotlib, Jason, Hermine, Olivier, Arock, Michel, Kluin-Nelemans, Hanneke C., Panse, Jens, Sperr, Wolfgang R., Valent, Peter, Reiter, Andreas, and Jawhar, Mohamad

Published
2023
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6. Prevalence of hypersensitivity reactions in various forms of mastocytosis: A pilot study of 2485 adult patients with mastocytosis collected in the ECNM registry

Author

Niedoszytko, Marek, primary, Gorska, Aleksandra, additional, Brockow, Knut, additional, Bonadonna, Patrizia, additional, Lange, Magdalena, additional, Kluin‐Nelemans, Hanneke, additional, Oude‐Elberink, Hanneke, additional, Sabato, Vito, additional, Shoumariyeh, Khalid, additional, von Bubnoff, Dagmar, additional, Müller, Sabine, additional, Illerhaus, Anja, additional, Doubek, Michael, additional, Angelova‐Fischer, Irena, additional, Hermine, Olivier, additional, Arock, Michel, additional, Elena, Chiara, additional, Malcovati, Luca, additional, Yavuz, Akif Selim, additional, Schug, Tanja Daniela, additional, Fortina, Anna Belloni, additional, Judit, Várkonyi, additional, Gotlib, Jason, additional, Panse, Jens, additional, Vucinic, Vladan, additional, Reiter, Andreas, additional, Schwaab, Juliana, additional, Triggiani, Massimo, additional, Mattsson, Mattias, additional, Breynaert, Christine, additional, Romantowski, Jan, additional, Zanotti, Roberta, additional, Olivieri, Elisa, additional, Zink, Alexander, additional, van de Ven, Annick, additional, Stefan, Alex, additional, Barete, Stephane, additional, Caroppo, Francesca, additional, Perkins, Cecelia, additional, Kennedy, Vanessa, additional, Christen, Deborah, additional, Jawhar, Mohamad, additional, Luebke, Johannes, additional, Parente, Roberta, additional, Levedahl, Kerstin, additional, Hadzijusufovic, Emir, additional, Hartmann, Karin, additional, Nedoszytko, Boguslaw, additional, Sperr, Wolfgang R., additional, and Valent, Peter, additional

Published
2024
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7. Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis

Author

Zanotti, Roberta, Bonifacio, Massimiliano, Lucchini, Giuseppe, Sperr, Wolfgang R., Scaffidi, Luigi, van Anrooij, Björn, Oude Elberink, Hanneke NC, Rossignol, Julien, Hermine, Olivier, Gorska, Aleksandra, Lange, Magdalena, Hadzijusufovic, Emir, Miething, Cornelius, Müller, Sabine, Perkins, Cecelia, Shomali, William, Elena, Chiara, Illerhaus, Anja, Jawhar, Mohamad, Parente, Roberta, Caroppo, Francesca, Solomianyi, Oleksii, Zink, Alexander, Mattsson, Mattias, Yavuz, Akif Selim, Panse, Jens, Varkonyi, Judit, Doubek, Michael, Sabato, Vito, Breynaert, Christine, Vucinic, Vladan, Schug, Tanja, Hägglund, Hans, Wortmann, Friederike, Brockow, Knut, Angelova-Fischer, Irena, Belloni Fortina, Anna, Triggiani, Massimo, Reiter, Andreas, Hartmann, Karin, Malcovati, Luca, Gotlib, Jason, Shoumariyeh, Khalid, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Bonadonna, Patrizia, and Valent, Peter

Published
2022
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8. Disease correlates and clinical relevance of hereditary α-tryptasemia in patients with systemic mastocytosis

Author

Sordi, Benedetta, Vanderwert, Fiorenza, Crupi, Francesca, Gesullo, Francesca, Zanotti, Roberta, Bonadonna, Patrizia, Crosera, Lara, Elena, Chiara, Fiorelli, Nicolas, Ferrari, Jacqueline, Grifoni, Federica, Sciumè, Mariarita, Parente, Roberta, Triggiani, Massimo, Palterer, Boaz, Mecheri, Valentina, Almerigogna, Fabio, Santi, Raffaella, Di Medio, Lisa, Brandi, Maria Luisa, Iorno, Maria Loredana, Ciardetti, Isabella, Bencini, Sara, Annunziato, Francesco, Mannarelli, Carmela, Pieri, Lisa, Guglielmelli, Paola, Mannelli, Francesco, and Vannucchi, Alessandro M.

Published
2022
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9. Defining a Severe Asthma Super-Responder: Findings from a Delphi Process

Author

Mansur, Adel, Detoraki, Aikaterini, Altraja, Alan, James, Alan, Nanzer-Kelly, Alexandra, Côté, Andréanne, Menzies-Gow, Andrew, Papaioannou, Andriana, Cheffins, Anne-Maree, Bourdin, Arnaud, Mahboub, Bassam, Lipworth, Brian, Celis-Preciado, Carlos Andrés, Torres-Duque, Carlos, Bucca, Caterina, Porsbjerg, Celeste, Ulrik, Charlotte, Corrigan, Chris, Taube, Christian, Farah, Claude, Katelaris, Constance, Langton, David, Ryan, Dermot, Larenas-Linnemann, Désirée, Zervas, Eleftherios, Heffler, Enrico, Hoyte, Flavia, Puggioni, Francesca, Christoff, George, Canonica, Giorgio Walter, Carpagnano, Giovanna Elisiana, Guida, Giuseppe, Katsoulotos, Gregory, Brusselle, Guy, Rupani, Hitashi, Jersmann, Hubertus, Clifton, Ian, Dhariwal, Jaideep, Fingleton, James, Duke, Jane, Rimmer, Janet, Douglass, Jo, Fonseca, João, van Boven, Job, Corless, John, Harrington, John, Maspero, Jorge, Miguel, José Luis, Pipatvech, Kanok, Kenny, Karrinda, Chapman, Kenneth, Kostikas, Konstantinos, Lehtimäki, Lauri, Chung, Li Ping, Heaney, Liam, Hang, Liang-Wen, Boulet, Louis-Philippe, Perez-de-Llano, Luis, Ricciardi, Luisa, Idrees, Majdy, Milanese, Manlio, Conte, Maria Elisabetta, Costantino, Maria Teresa, Siyue, Mariko Koh, Fitzgerald, Mark, Hew, Mark, Peters, Matthew, Tsai, Ming-Ju, Patel, Mitesh, Khan, Mohammad Hashim, Sadatsafavi, Mohsen, Al-Ahmad, Mona, Yacoub, Mona-Rita, De Gennaro, Mónica, Radhakrishna, Naghmeh, Hanania, Nicola Alexander, Papadopoulos, Nikolaos, Lugogo, Njira, Linaker, Norma, Crimi, Nunzio, Dennison, Paddy, Nair, Parameswaran, Mitchell, Patrick David, O’Byrne, Paul, Pfeffer, Paul, Kauppi, Paula, Hughes, Pauline, Middleton, Peter, Wark, Peter, Bardin, Philip, Fu, Pin-Kuei, Akuthota, Praveen, Chaudhuri, Rekha, Campos, Ricardo, Al-Lehebi, Riyard, Parente, Roberta, Francisco, Rovira, Wenzel, Sally, Pierachille, Santus, Pawar, Shrikant, Loukides, Stelios, Fowler, Stephen, Mackenzie, Tara, Brown, Thomas, Lee Tan, Tze, Björnsdóttir, Unnur, McDonald, Vanessa, Lawriwskyj, Veronica, Backer, Vibeke, Vasileva, Violina, Chien, Ying-Chun, Harrington, Zinta, Upham, John W., Le Lievre, Chantal, Jackson, David J., Masoli, Matthew, Wechsler, Michael E., and Price, David B.

Published
2021
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10. Severe asthma: One disease and multiple definitions

Author

Guarnieri, Gabriella, Patella, Vincenzo, Maria Pia, Foschino Barbaro, Carpagnano, Giovanna Elisiana, Colle, Anna del, Scioscia, Giulia, Gerolamo, Pelaia, Puggioni, Francesca, Racca, Francesca, Favero, Elisabetta, Iannacone, Sandra, Savi, Eleonora, Montagni, Marcello, Camiciottoli, Gianna, Allegrini, Chiara, Lombardi, Carlo, Spadaro, Giuseppe, Detoraki, Caterina, Menzella, Francesco, Galeone, Carla, Ruggiero, Patrizia, Yacoub, Monna Rita, Berti, Alvise, Scichilone, Nicola, Durante, Carmen, Costantino, Maria Teresa, Roncallo, Chiara, Braschi, Mariachiara, D’Adda, Alice, Ridolo, Erminia, Triggiani, Massimo, Parente, Roberta, Maria, D’Amato, Verrillo, Maria Vittoria, Rolla, Giovanni, Brussino, Luisa, Frazzetto, Agata Valentina, Cristina, Zappa Maria, Lilli, Marianna, Crimi, Nunzio, Bonavia, Marco, Corsico, Angelo Guido, Grosso, Amelia, Del Giacco, Stefano, Deidda, Margherita, Ricciardi, Luisa, Isola, Stefania, Cicero, Francesca, Amato, Giuliana, Vita, Federica, Spanevello, Antonio, Pignatti, Patrizia, Cherubino, Francesca, Visca, Dina, Massimo Ricciardolo, Fabio Luigi, Anna Carriero, Vitina Maria, Bertolini, Francesca, Santus, Pierachille, Barlassina, Roberta, Airoldi, Andrea, Guida, Giuseppe, Eleonora, Nucera, Aruanno, Arianna, Rizzi, Angela, Caruso, Cristiano, Colantuono, Stefania, Senna, Gianenrico, Caminati, Marco, Arcolaci, Alessandra, Vianello, Andrea, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Centanni, Stefano, Luraschi, Simone, Ruggeri, Silvia, Rinaldo, Rocco, Parazzini, Elena, Calabrese, Cecilia, Flora, Martina, Cosmi, Lorenzo, Di Pietro, Linda, Maggi, Enrico, Pini, Laura, Macchia, Luigi, Di Bona, Danilo, Richeldi, Luca, Condoluci, Carola, Fuso, Leonello, Bonini, Matteo, Farsi, Alessandro, Carli, Giulia, Montuschi, Paolo, Santini, Giuseppe, Conte, Maria Elisabetta, Turchet, Elisa, Barbetta, Carlo, Mazza, Francesco, D’Alo, Simona, Pucci, Stefano, Caiaffa, Maria Filomena, Minenna, Elena, D'Elia, Luciana, Pasculli, Carlo, Viviano, Vittorio, Tarsia, Paolo, Rolo, Joyce, Di Proietto, Mariacarmela, Lo Cicero, Salvatore, Bagnasco, Diego, Paggiaro, Pierluigi, Latorre, Manuela, Folli, Chiara, Testino, Elisa, Bassi, Arianna, Milanese, Manlio, Heffler, Enrico, Manfredi, Andrea, Riccio, Anna Maria, De Ferrari, Laura, Blasi, Francesco, Canevari, Rikki Frank, Canonica, Giorgio Walter, and Passalacqua, Giovanni

Published
2021
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11. Clinical Impact of Skin Lesions in Mastocytosis: A Multicenter Study of the European Competence Network on Mastocytosis

Author

Aberer, Elisabeth, Sperr, Wolfgang R., Bretterklieber, Agnes, Avian, Alexander, Hadzijusufovic, Emir, Kluin-Nelemans, Hanneke C., Oude Elberink, Hanneke, van Anrooij, Björn, Niedoszytko, Marek, Lange, Magdalena, Górska, Aleksandra, Elena, Chiara, Brazzelli, Valeria, Belloni Fortina, Anna, Caroppo, Francesca, Hartmann, Karin, Illerhaus, Anja, Reiter, Andreas, Jawhar, Mohamad, Bonadonna, Patrizia, Zanotti, Roberta, Triggiani, Massimo, Parente, Roberta, Gotlib, Jason, Doubek, Michael, von Bubnoff, Nikolas, Fuchs, David, Sabato, Vito, Brockow, Knut, Jäkel, Nadja, Panse, Jens, and Valent, Peter

Published
2021
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13. Scoring the Risk of Having Systemic Mastocytosis in Adult Patients with Mastocytosis in the Skin

Author

Fuchs, David, Kilbertus, Alex, Kofler, Karin, von Bubnoff, Nikolas, Shoumariyeh, Khalid, Zanotti, Roberta, Bonadonna, Patrizia, Scaffidi, Luigi, Doubek, Michael, Elberink, Hanneke Oude, Span, Lambert F.R., Hermine, Olivier, Elena, Chiara, Benvenuti, Pietro, Yavuz, Akif Selim, Brockow, Knut, Zink, Alexander, Aberer, Elisabeth, Gorska, Aleksandra, Romantowski, Jan, Hadzijusufovic, Emir, Fortina, Anna Belloni, Caroppo, Francesca, Perkins, Cecelia, Illerhaus, Anja, Panse, Jens, Vucinic, Vladan, Jawhar, Mohamad, Sabato, Vito, Triggiani, Massimo, Parente, Roberta, Bergström, Anna, Breynaert, Christine, Gotlib, Jason, Reiter, Andreas, Hartmann, Karin, Niedoszytko, Marek, Arock, Michel, Kluin-Nelemans, Hanneke C., Sperr, Wolfgang R., Greul, Rosemarie, and Valent, Peter

Published
2021
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14. Economic impact of mepolizumab in uncontrolled severe eosinophilic asthma, in real life

Author

Guarnieri, Gabriella, Patella, Vincenzo, Maria Pia, Foschino Barbaro, Carpagnano, Elisiana, Colle, Anna del, Scioscia, Giulia, Gerolamo, Pelaia, Paggiaro, Pierluigi, Latorre, Manuela, Puggioni, Francesca, Racca, Francesca, Favero, Elisabetta, Iannacone, Sandra, Savi, Eleonora, Montagni, Marcello, Camiciottoli, Gianna, Allegrini, Chiara, Spadaro, Giuseppe, Detoraki, Caterina, Galeone, Carla, Ruggiero, Patrizia, Yacoub, Monna Rita, Berti, Alvise, Colombo, Gisella, Scichilone, Nicola, Durante, Carmen, Costantino, Maria Teresa, Roncallo, Chiara, Braschi, Mariachiara, Blasi, Francesco, D'Adda, Alice, Ridolo, Erminia, Triggiani, Massimo, Parente, Roberta, Maria, D'Amato, Verrillo, Maria Vittoria, Cristina, Zappa Maria, Lilli, Marianna, Crimi, Nunzio, Bonavia, Marco, Corsico, Angelo Guido, Grosso, Amelia, Del Giacco, Stefano, Deidda, Margherita, Ricciardi, Luisa, Isola, Stefania, Cicero, Francesca, Amato, Giuliana, Vita, Federica, Spanevello, Antonio, Pignatti, Patrizia, Cherubino, Francesca, Visca, Dina, Aletti, Eleonora, Massimo Ricciardolo, Fabio Luigi, Anna Carriero, Vitina Maria, Bertolini, Francesca, Santus, Pierachille, Barlassina, Roberta, Airoldi, Andrea, Guida, Giuseppe, Eleonora, Nucera, Aruanno, Arianna, Rizzi, Angela, Caruso, Cristiano, Colantuono, Stefania, Arcolaci, Alessandra, Vianello, Andrea, Bianchi, Fulvia Chieco, Marchi, Maria Rita, Centanni, Stefano, Luraschi, Simone, Ruggeri, Silvia, Rinaldo, Rocco, Parazzini, Elena, Calabrese, Cecilia, Flora, Martina, Cosmi, Lorenzo, Di Pietro, Linda, Maggi, Enrico, Pini, Laura, Macchia, Luigi, Di Bona, Danilo, Richeldi, Luca, Condoluci, Carola, Fuso, Leonello, Bonini, Matteo, Farsi, Alessandro, Carli, Giulia, Montuschi, Paolo, Santini, Giuseppe, Conte, Maria Elisabetta, Turchet, Elisa, Barbetta, Carlo, Mazza, Francesco, D'Alo, Simona, Pucci, Stefano, Caiaffa, Maria Filomena, Minenna, Elena, D'Elia, Luciana, Pasculli, Carlo, Viviano, Vittorio, Tarsia, Paolo, Rolo, Joyce, Di Proietto, Mariacarmela, Lo Cicero, Salvatore, Bagnasco, Diego, Povero, Massimiliano, Pradelli, Lorenzo, Brussino, Luisa, Rolla, Giovanni, Caminati, Marco, Menzella, Francesco, Heffler, Enrico, Canonica, Giorgio Walter, Senna, Gianenrico, Milanese, Manlio, Lombardi, Carlo, Bucca, Caterina, Manfredi, Andrea, Canevari, Rikki Frank, and Passalacqua, Giovanni

Published
2021
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15. Asthma management, focused on the use of oral corticosteroids: the opinions of Italian asthmatic patients.

Author

Latorre, Manuela, Rizzi, Angela, Paggiaro, Pierluigi, Baiardini, Ilaria, Bagnasco, Diego, Del Giacco, Stefano, Lombardi, Carlo, Patella, Vincenzo, Nucera, Eleonora, Parente, Roberta, Paoletti, Giovanni, Pini, Laura, Ridolo, Erminia, Senna, Gianenrico, Blasi, Francesco, and Canonica, Giorgio Walter

Subjects
PATIENTS' attitudes, ORAL drug administration, QUALITY of life, DELAYED diagnosis, ASTHMA
Abstract

Objective: Patients' perceptions of asthma symptoms, and attitudes regarding diagnosis and management, can affect their ability to reach good asthma control. The aim of the study was to explore patients' perceptions of asthma management, with focus on treatment with oral corticosteroids (OCS). Methods: A DOXAPHARMA survey was conducted. A questionnaire with 46 multiple choice questions was completed by 50 patients with severe uncontrolled asthma, and 258 with mild–moderate controlled or partly controlled asthma. Participants were representative of Italian asthmatic patients—with medium age, long asthma duration, delayed diagnosis, poor asthma control, and frequent exacerbations. Results: Many asthmatics reported inadequate pharmacologic treatment. The majority but not all patients regularly used ICS/LABA. Oral treatment was common, mainly with OCS, particularly in severe asthmatics. One-fourth of patients did not regularly use inhaled therapy, and adherence was poor, resulting in frequent OCS use to treat exacerbations, which were common in mild–moderate cases. Patients were fairly satisfied with asthma therapies, but many had concerns about long-term corticosteroid use. Patients complained about poor management of comorbidities associated with asthma and OCS use, but were generally satisfied with their patient/doctor relationships. Many patients failed to achieve optimal health-related quality of life (HRQoL), mainly those with severe asthma who used OCS treatment and emphasized how OCS therapy impacted QoL. Conclusions: The survey results confirmed many problems related to mild–moderate and severe asthma management in Italy and highlighted the overuse of OCS rather than more effective and safe treatments, which had strong negative effects on HRQoL. [ABSTRACT FROM AUTHOR]

Published
2024
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16. Oral CorticoSteroid sparing with biologics in severe asthma: A remark of the Severe Asthma Network in Italy (SANI)

Author

Canonica, Giorgio Walter, Blasi, Francesco, Paggiaro, Pierluigi, Senna, Gianenrico, Passalacqua, Giovanni, Spanevello, Antonio, Aliberti, Stefano, Bagnasco, Diego, Bonavia, Marco, Bonini, Matteo, Brussino, Luisa, Bucca, Caterina, Caiaffa, Maria F., Calabrese, Cecilia, Camiciottoli, Gianna, Caminati, Marco, Carpagnano, Giovanna E., Caruso, Cristiano, Centanni, Stefano, Conte, Maria E., Corsico, Angelo G., Cosmi, Lorenzo, Costantino, Maria T., Crimi, Nunzio, D’Alò, Simona, D'Amato, Maria, Del Giacco, Stefano, Farsi, Alessandro, Favero, Elisabetta, Foschino Barbaro, Maria P., Guarnieri, Gabriella, Guida, Giuseppe, Latorre, Manuela, Lo Cicero, Salvatore, Lombardi, Carlo, Macchia, Luigi, Mazza, Francesco, Menzella, Francesco, Milanese, Manlio, Montagni, Marcello, Montuschi, Paolo, Nucera, Eleonora, Parente, Roberta, Patella, Vincenzo, Pelaia, Girolamo, Pini, Laura, Puggioni, Francesca, Ricciardi, Luisa, Ricciardolo, Fabio L.M., Richeldi, Luca, Ridolo, Erminia, Rolla, Giovanni, Santus, Pierachille, Scichilone, Nicola, Spadaro, Giuseppe, Vianello, Andrea, Viviano, Vittorio, Yacoub, Mona R., Zappa, Maria C., and Heffler, Enrico

Published
2020
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17. Quality of life in patients with allergic and immunologic skin diseases: in the eye of the beholder

Author

Di Agosta, Ester, Salvati, Lorenzo, Corazza, Monica, Baiardini, Ilaria, Ambrogio, Francesca, Angileri, Luisa, Antonelli, Elettra, Belluzzo, Federica, Bonamonte, Domenico, Bonzano, Laura, Brancaccio, Raffaele, Custurone, Paolo, De Marco, Aurora, Detoraki, Aikaterini, Di Guida, Adriana, Di Leo, Elisabetta, Fantò, Marta, Fassio, Filippo, Ferrucci, Silvia Mariel, Foti, Caterina, Gallo, Rosella, Gatta, Alessia, Guarneri, Fabrizio, Guidolin, Lucia, Hansel, Katharina, Lamacchia, Donatella, Lombardo, Carla, Minciullo, Paola Lucia, Napolitano, Maddalena, Pannofino, Alessandro, Paravisi, Andrea, Parente, Roberta, Passante, Maria, Patruno, Cataldo, Peroni, Diego, Quecchia, Cristina, Schettini, Natale, Spadaro, Giuseppe, Stingeni, Luca, Tarrini, Daniele, Tramontana, Marta, Nettis, Eustachio, and Rossi, Oliviero

Published
2021
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18. International prognostic scoring system for mastocytosis (IPSM): a retrospective cohort study

Author

Sperr, Wolfgang R, Kundi, Michael, Alvarez-Twose, Ivan, van Anrooij, Bjorn, Oude Elberink, Joanna N G, Gorska, Aleksandra, Niedoszytko, Marek, Gleixner, Karoline V, Hadzijusufovic, Emir, Zanotti, Roberta, Bonadonna, Patrizia, Bonifacio, Massimiliano, Perkins, Cecelia, Illerhaus, Anja, Elena, Chiara, Merante, Serena, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Parente, Roberta, Jawhar, Mohamad, Belloni Fortina, Anna, Caroppo, Francesca, Brockow, Knut, Zink, Alexander, Fuchs, David, Kilbertus, Alex J, Yavuz, Akif Selim, Doubek, Michael, Hägglund, Hans, Panse, Jens, Sabato, Vito, Bretterklieber, Agnes, Niederwieser, Dietger, Breynaert, Christine, Hartmann, Karin, Triggiani, Massimo, Nedoszytko, Boguslaw, Reiter, Andreas, Orfao, Alberto, Hermine, Olivier, Gotlib, Jason, Arock, Michel, Kluin-Nelemans, Hanneke C, and Valent, Peter

Published
2019
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20. Prognostic impact of eosinophils in mastocytosis: analysis of 2350 patients collected in the ECNM Registry

Author

Kluin-Nelemans, Hanneke C., Reiter, Andreas, Illerhaus, Anja, van Anrooij, Bjorn, Hartmann, Karin, Span, Lambertus F. R., Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Parente, Roberta, Triggiani, Massimo, Schwaab, Juliana, Jawhar, Mohamad, Caroppo, Francesca, Fortina, Anna Belloni, Brockow, Knut, Zink, Alexander, Fuchs, David, Kilbertus, Alex, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hagglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Niederwieser, Dietger, Breynaert, Christine, Várkonyi, Judit, Kennedy, Vanessa, Lortholary, Olivier, Jakob, Thilo, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Gotlib, Jason, Valent, Peter, and Sperr, Wolfgang R.

Published
2020
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21. The Severe Asthma Network in Italy: Findings and Perspectives

Author

Barbuto, Sarah, Bradicich, Matteo, Camiciottoli, Gianna, Caminati, Marco, Colombo, Giselda, Costantino, Maria Teresa, Crimi, Nunzio, Crivellaro, Mariangiola, D’Adda, Alice, D’Amato, Mariella, Favero, Elisabetta, Foschino, Maria Pia, Galeone, Carla, Guarnieri, Gabriella, Lombardi, Carlo, Parente, Roberta, Passalacqua, Giovanni, Patella, Vincenzo, Puggioni, Francesca, Ridolo, Erminia, Rolla, Giovanni, Savi, Eleonora, Scichilone, Nicola, Solidoro, Paolo, Spadaro, Giuseppe, Heffler, Enrico, Blasi, Francesco, Latorre, Manuela, Menzella, Francesco, Paggiaro, Pierluigi, Pelaia, Girolamo, Senna, Gianenrico, and Canonica, Giorgio Walter

Published
2019
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22. The Data Registry of the European Competence Network on Mastocytosis (ECNM): Set Up, Projects, and Perspectives

Author

Valent, Peter, Oude Elberink, Joanna N.G., Gorska, Aleksandra, Lange, Magdalena, Zanotti, Roberta, van Anrooij, Björn, Bonifacio, Massimiliano, Bonadonna, Patrizia, Gleixner, Karoline V., Hadzijusufovic, Emir, Perkins, Cecelia, Hartmann, Karin, Illerhaus, Anja, Merante, Serena, Elena, Chiara, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Parente, Roberta, Triggiani, Massimo, Schwaab, Juliana, Jawhar, Mohamad, Caroppo, Francesca, Fortina, Anna Belloni, Brockow, Knut, David Fuchs, Greul, Rosemarie, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hagglund, Hans, Panse, Jens, Sabato, Vito, Aberer, Elisabeth, Al-Ali, Haifa Kathrin, Morren, Marie-Anne, Varkonyi, Judit, Zink, Alexander, Niedoszytko, Marek, Niederwieser, Dietger, Malcovati, Luca, Reiter, Andreas, Kennedy, Vanessa, Gotlib, Jason, Lortholary, Olivier, Hermine, Olivier, Arock, Michel, Kluin-Nelemans, Hanneke, and Sperr, Wolfgang R.

Published
2019
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23. Chronic Urticaria Patient Perspective (CUPP): The First Validated Tool for Assessing Quality of Life in Clinical Practice

Author

Baiardini, Ilaria, Braido, Fulvio, Molinengo, Giorgia, Caminati, Marco, Costantino, MariaTeresa, Cristaudo, Antonio, Crivellaro, Mariangiola, Ferrucci, Silvia Mariel, Gallo, Rosella, Giorgis, Veronica, Legori, Agostina, Loera, Barbara, Martignago, Irene, Marzano, Angelo Valerio, Morrone, Aldo, Parente, Roberta, Parodi, Aurora, Parolo, Anna, Peveri, Silvia, Pigatto, Paolo, Radice, Anna, Ridolo, Erminia, Rolla, Giovanni, Roncallo, Chiara, Rossi, Oliviero, Savi, Eleonora, Senna, Gianenrico, Triggiani, Massimo, and Canonica, Giorgio Walter

Published
2018
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24. Multicentric Observational Study on Safety and Tolerability of COVID-19 Vaccines in Patients with Angioedema with C1 Inhibitor Deficiency: Data from Italian Network on Hereditary and Acquired Angioedema (ITACA)

Author

Parente, Roberta, primary, Sartorio, Silvio, additional, Brussino, Luisa, additional, De Pasquale, Tiziana, additional, Zoli, Alessandra, additional, Agolini, Stefano, additional, Di Agosta, Ester, additional, Quattrocchi, Paolina, additional, Borrelli, Paolo, additional, Bignardi, Donatella, additional, Petraroli, Angelica, additional, Senter, Riccardo, additional, Popescu Janu, Valentina, additional, Cogliati, Chiara, additional, Guarino, Maria Domenica, additional, Rossi, Oliviero, additional, Firinu, Davide, additional, Pucci, Stefano, additional, Spadaro, Giuseppe, additional, Triggiani, Massimo, additional, Cancian, Mauro, additional, and Zanichelli, Andrea, additional

Published
2023
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27. Identifying and Managing Those at Risk for Vaccine-Related Allergy and Anaphylaxis

Author

Stone, Cosby A., Garvey, Lene H., Nasser, Shuaib, Lever, Charley, Triggiani, Massimo, Parente, Roberta, Phillips, Elizabeth J., Stone, Cosby A., Garvey, Lene H., Nasser, Shuaib, Lever, Charley, Triggiani, Massimo, Parente, Roberta, and Phillips, Elizabeth J.

Abstract

Immediate hypersensitivity reactions to vaccines, the most severe of which is anaphylaxis, are uncommon events occurring in fewer than 1 in a million doses administered. These reactions are infrequently immunoglobulin E–mediated. Because they are unlikely to recur, a reaction to a single dose of a vaccine is rarely a contraindication to redosing. This narrative review article contextualizes the recent knowledge we have gained from the coronavirus 2019 (COVID-19) pandemic rollout of the new mRNA platform with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines within the much broader context of what is known about immediate reactions to other vaccinations of routine and global importance. We focus on what is known about evidence-based approaches to diagnosis and management and what is new in our understanding of mechanisms of immediate vaccine reactions. Specifically, we review the epidemiology of immediate hypersensitivity vaccine reactions, differential diagnosis for immune-mediated and nonimmune reaction clinical phenotypes, including how to recognize immunization stress–related responses. In addition, we highlight what is known about mechanisms and review the rare but important contribution of excipient allergies and specifically when to consider testing for them as well as other key features that contribute to safe evaluation and management.

Published
2023

28. Disease correlates and clinical relevance of hereditary α-tryptasemia in patients with systemic mastocytosis

Author

Sordi, Benedetta, primary, Vanderwert, Fiorenza, additional, Crupi, Francesca, additional, Gesullo, Francesca, additional, Zanotti, Roberta, additional, Bonadonna, Patrizia, additional, Crosera, Lara, additional, Elena, Chiara, additional, Fiorelli, Nicolas, additional, Ferrari, Jacqueline, additional, Grifoni, Federica, additional, Sciumè, Mariarita, additional, Parente, Roberta, additional, Triggiani, Massimo, additional, Palterer, Boaz, additional, Mecheri, Valentina, additional, Almerigogna, Fabio, additional, Santi, Raffaella, additional, Di Medio, Lisa, additional, Brandi, Maria Luisa, additional, Iorno, Maria Loredana, additional, Ciardetti, Isabella, additional, Bencini, Sara, additional, Annunziato, Francesco, additional, Mannarelli, Carmela, additional, Pieri, Lisa, additional, Guglielmelli, Paola, additional, Mannelli, Francesco, additional, and Vannucchi, Alessandro M., additional

Published
2023
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31. Real-World Management of Advanced Systemic Mastocytosis Treated with Midostaurin: Analysis of Patients Who Completed 12 Months of Follow-up from an Italian Observational Study (OVIDIO)

Author

Papayannidis, Cristina, primary, Mannelli, Francesco, additional, Crosera, Lara, additional, Parente, Roberta, additional, Romano, Alessandra, additional, Rondoni, Michela, additional, Elena, Chiara, additional, Criscuolo, Marianna, additional, Di Renzo, Nicola, additional, Giona, Fiorina, additional, Pane, Fabrizio, additional, Cilloni, Daniela, additional, Elli, Elena Maria, additional, Miglino, Maurizio, additional, Mazza, Patrizio, additional, Malato, Alessandra, additional, Pochintesta, Lara, additional, Bini, Roberta, additional, Valsecchi, Diletta, additional, and Grifoni, Federica Irene, additional

Published
2022
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38. Vascular endothelial growth factors and angiopoietins as new players in mastocytosis. (VARRICCHI AUTORE CORRISPONDENTE)

Author

Marcella, Simone, Petraroli, Angelica, Braile, Mariantonia, Parente, Roberta, Ferrara, Anne Lise, Galdiero, Maria Rosaria, Modestino, Luca, Cristinziano, Leonardo, Rossi, Francesca Wanda, Varricchi, Gilda, Triggiani, Massimo, de Paulis, Amato, Spadaro, Giuseppe, Loffredo, Stefania, Marcella, Simone, Petraroli, Angelica, Braile, Mariantonia, Parente, Roberta, Ferrara, Anne Lise, Galdiero, Maria Rosaria, Modestino, Luca, Cristinziano, Leonardo, Rossi, Francesca Wanda, Varricchi, Gilda, Triggiani, Massimo, de Paulis, Amato, Spadaro, Giuseppe, and Loffredo, Stefania

Subjects
Vascular endothelial growth factors, Mastocytosi, Tryptase, Angiopoietin, Mast cell, Stem cell factor
Abstract

Mastocytosis is a disorder characterized by the abnormal proliferation and/or accumulation of mast cells in different organs. More than 90% of patients with systemic mastocytosis have a gain-of-function mutation in codon 816 of the KIT receptor on mast cells (MCs). The symptoms of mastocytosis patients are related to the MC-derived mediators that exert local and distant effects. MCs produce angiogenic and lymphangiogenic factors, including vascular endothelial growth factors (VEGFs) and angiopoietins (ANGPTs). Serum concentrations of VEGF-A, VEGF-C, VEGF-D, ANGPT1 and ANGPT2 were determined in 64 mastocytosis patients and 64 healthy controls. Intracellular concentrations and spontaneous release of these mediators were evaluated in the mast cell lines ROSAKIT WT and ROSA KIT D816V and in human lung mast cells (HLMCs). VEGF-A, ANGPT1, ANGPT2 and VEGF-C concentrations were higher in mastocytosis patients compared to controls. The VEGF-A, ANGPT2 and VEGF-C concentrations were correlated with the symptom severity. ANGPT1 concentrations were increased in all patients compared to controls. ANGPT2 levels were correlated with severity of clinical variants and with tryptase levels. VEGF-A, ANGPT1 and VEGF-C did not differ between indolent and advanced mastocytosis. ROSAKIT WT, ROSAKIT D816V and HLMCs contained and spontaneously released VEGFs and ANGPTs. Serum concentrations of VEGFs and ANGPTs are altered in mastocytosis patients.

Published
2021

40. Screening for Hereditary Alpha-Tryptasemia in Subjects with Systemic Mastocytosis (SM) and Non-SM Mast Cell Activation Symptoms

Author

Vanderwert, Fiorenza Irushani, primary, Sordi, Benedetta, additional, Mannelli, Francesco, additional, Palterer, Boaz, additional, Gesullo, Francesca, additional, Mecheri, Valentina, additional, Santi, Raffaella, additional, Mannarelli, Carmela, additional, Crupi, Francesca, additional, Zanotti, Roberta, additional, Grifoni, Federica Irene, additional, Elena, Chiara, additional, Parente, Roberta, additional, Triggiani, Massimo, additional, Lisa, Pieri, additional, Almerigogna, Fabio, additional, Guglielmelli, Paola, additional, and Vannucchi, Alessandro, additional

Published
2021
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41. Defining a Severe Asthma Super-Responder: Findings from a Delphi Process

Author

Upham, John W., primary, Le Lievre, Chantal, additional, Jackson, David J., additional, Masoli, Matthew, additional, Wechsler, Michael E., additional, Price, David B., additional, Mansur, Adel, additional, Detoraki, Aikaterini, additional, Altraja, Alan, additional, James, Alan, additional, Nanzer-Kelly, Alexandra, additional, Côté, Andréanne, additional, Menzies-Gow, Andrew, additional, Papaioannou, Andriana, additional, Cheffins, Anne-Maree, additional, Bourdin, Arnaud, additional, Mahboub, Bassam, additional, Lipworth, Brian, additional, Celis-Preciado, Carlos Andrés, additional, Torres-Duque, Carlos, additional, Bucca, Caterina, additional, Porsbjerg, Celeste, additional, Ulrik, Charlotte, additional, Corrigan, Chris, additional, Taube, Christian, additional, Farah, Claude, additional, Katelaris, Constance, additional, Langton, David, additional, Ryan, Dermot, additional, Larenas-Linnemann, Désirée, additional, Zervas, Eleftherios, additional, Heffler, Enrico, additional, Hoyte, Flavia, additional, Puggioni, Francesca, additional, Christoff, George, additional, Canonica, Giorgio Walter, additional, Carpagnano, Giovanna Elisiana, additional, Guida, Giuseppe, additional, Katsoulotos, Gregory, additional, Brusselle, Guy, additional, Rupani, Hitashi, additional, Jersmann, Hubertus, additional, Clifton, Ian, additional, Dhariwal, Jaideep, additional, Fingleton, James, additional, Duke, Jane, additional, Rimmer, Janet, additional, Douglass, Jo, additional, Fonseca, João, additional, van Boven, Job, additional, Corless, John, additional, Harrington, John, additional, Maspero, Jorge, additional, Miguel, José Luis, additional, Pipatvech, Kanok, additional, Kenny, Karrinda, additional, Chapman, Kenneth, additional, Kostikas, Konstantinos, additional, Lehtimäki, Lauri, additional, Chung, Li Ping, additional, Heaney, Liam, additional, Hang, Liang-Wen, additional, Boulet, Louis-Philippe, additional, Perez-de-Llano, Luis, additional, Ricciardi, Luisa, additional, Idrees, Majdy, additional, Milanese, Manlio, additional, Conte, Maria Elisabetta, additional, Costantino, Maria Teresa, additional, Siyue, Mariko Koh, additional, Fitzgerald, Mark, additional, Hew, Mark, additional, Peters, Matthew, additional, Tsai, Ming-Ju, additional, Patel, Mitesh, additional, Khan, Mohammad Hashim, additional, Sadatsafavi, Mohsen, additional, Al-Ahmad, Mona, additional, Yacoub, Mona-Rita, additional, De Gennaro, Mónica, additional, Radhakrishna, Naghmeh, additional, Hanania, Nicola Alexander, additional, Papadopoulos, Nikolaos, additional, Lugogo, Njira, additional, Linaker, Norma, additional, Crimi, Nunzio, additional, Dennison, Paddy, additional, Nair, Parameswaran, additional, Mitchell, Patrick David, additional, O’Byrne, Paul, additional, Pfeffer, Paul, additional, Kauppi, Paula, additional, Hughes, Pauline, additional, Middleton, Peter, additional, Wark, Peter, additional, Bardin, Philip, additional, Fu, Pin-Kuei, additional, Akuthota, Praveen, additional, Chaudhuri, Rekha, additional, Campos, Ricardo, additional, Al-Lehebi, Riyard, additional, Parente, Roberta, additional, Francisco, Rovira, additional, Wenzel, Sally, additional, Pierachille, Santus, additional, Pawar, Shrikant, additional, Loukides, Stelios, additional, Fowler, Stephen, additional, Mackenzie, Tara, additional, Brown, Thomas, additional, Lee Tan, Tze, additional, Björnsdóttir, Unnur, additional, McDonald, Vanessa, additional, Lawriwskyj, Veronica, additional, Backer, Vibeke, additional, Vasileva, Violina, additional, Chien, Ying-Chun, additional, and Harrington, Zinta, additional

Published
2021
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42. Severe asthma: One disease and multiple definitions

Author

Bagnasco, Diego, primary, Paggiaro, Pierluigi, additional, Latorre, Manuela, additional, Folli, Chiara, additional, Testino, Elisa, additional, Bassi, Arianna, additional, Milanese, Manlio, additional, Heffler, Enrico, additional, Manfredi, Andrea, additional, Riccio, Anna Maria, additional, De Ferrari, Laura, additional, Blasi, Francesco, additional, Canevari, Rikki Frank, additional, Canonica, Giorgio Walter, additional, Passalacqua, Giovanni, additional, Guarnieri, Gabriella, additional, Patella, Vincenzo, additional, Maria Pia, Foschino Barbaro, additional, Carpagnano, Giovanna Elisiana, additional, Colle, Anna del, additional, Scioscia, Giulia, additional, Gerolamo, Pelaia, additional, Puggioni, Francesca, additional, Racca, Francesca, additional, Favero, Elisabetta, additional, Iannacone, Sandra, additional, Savi, Eleonora, additional, Montagni, Marcello, additional, Camiciottoli, Gianna, additional, Allegrini, Chiara, additional, Lombardi, Carlo, additional, Spadaro, Giuseppe, additional, Detoraki, Caterina, additional, Menzella, Francesco, additional, Galeone, Carla, additional, Ruggiero, Patrizia, additional, Yacoub, Monna Rita, additional, Berti, Alvise, additional, Scichilone, Nicola, additional, Durante, Carmen, additional, Costantino, Maria Teresa, additional, Roncallo, Chiara, additional, Braschi, Mariachiara, additional, D’Adda, Alice, additional, Ridolo, Erminia, additional, Triggiani, Massimo, additional, Parente, Roberta, additional, Maria, D’Amato, additional, Verrillo, Maria Vittoria, additional, Rolla, Giovanni, additional, Brussino, Luisa, additional, Frazzetto, Agata Valentina, additional, Cristina, Zappa Maria, additional, Lilli, Marianna, additional, Crimi, Nunzio, additional, Bonavia, Marco, additional, Corsico, Angelo Guido, additional, Grosso, Amelia, additional, Del Giacco, Stefano, additional, Deidda, Margherita, additional, Ricciardi, Luisa, additional, Isola, Stefania, additional, Cicero, Francesca, additional, Amato, Giuliana, additional, Vita, Federica, additional, Spanevello, Antonio, additional, Pignatti, Patrizia, additional, Cherubino, Francesca, additional, Visca, Dina, additional, Massimo Ricciardolo, Fabio Luigi, additional, Anna Carriero, Vitina Maria, additional, Bertolini, Francesca, additional, Santus, Pierachille, additional, Barlassina, Roberta, additional, Airoldi, Andrea, additional, Guida, Giuseppe, additional, Eleonora, Nucera, additional, Aruanno, Arianna, additional, Rizzi, Angela, additional, Caruso, Cristiano, additional, Colantuono, Stefania, additional, Senna, Gianenrico, additional, Caminati, Marco, additional, Arcolaci, Alessandra, additional, Vianello, Andrea, additional, Bianchi, Fulvia Chieco, additional, Marchi, Maria Rita, additional, Centanni, Stefano, additional, Luraschi, Simone, additional, Ruggeri, Silvia, additional, Rinaldo, Rocco, additional, Parazzini, Elena, additional, Calabrese, Cecilia, additional, Flora, Martina, additional, Cosmi, Lorenzo, additional, Di Pietro, Linda, additional, Maggi, Enrico, additional, Pini, Laura, additional, Macchia, Luigi, additional, Di Bona, Danilo, additional, Richeldi, Luca, additional, Condoluci, Carola, additional, Fuso, Leonello, additional, Bonini, Matteo, additional, Farsi, Alessandro, additional, Carli, Giulia, additional, Montuschi, Paolo, additional, Santini, Giuseppe, additional, Conte, Maria Elisabetta, additional, Turchet, Elisa, additional, Barbetta, Carlo, additional, Mazza, Francesco, additional, D’Alo, Simona, additional, Pucci, Stefano, additional, Caiaffa, Maria Filomena, additional, Minenna, Elena, additional, D'Elia, Luciana, additional, Pasculli, Carlo, additional, Viviano, Vittorio, additional, Tarsia, Paolo, additional, Rolo, Joyce, additional, Di Proietto, Mariacarmela, additional, and Lo Cicero, Salvatore, additional

Published
2021
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43. Refined diagnostic criteria for bone marrow mastocytosis: a proposal of the European competence network on mastocytosis

Author

Zanotti, Roberta, primary, Bonifacio, Massimiliano, additional, Lucchini, Giuseppe, additional, Sperr, Wolfgang R., additional, Scaffidi, Luigi, additional, van Anrooij, Björn, additional, Oude Elberink, Hanneke NC, additional, Rossignol, Julien, additional, Hermine, Olivier, additional, Gorska, Aleksandra, additional, Lange, Magdalena, additional, Hadzijusufovic, Emir, additional, Miething, Cornelius, additional, Müller, Sabine, additional, Perkins, Cecelia, additional, Shomali, William, additional, Elena, Chiara, additional, Illerhaus, Anja, additional, Jawhar, Mohamad, additional, Parente, Roberta, additional, Caroppo, Francesca, additional, Solomianyi, Oleksii, additional, Zink, Alexander, additional, Mattsson, Mattias, additional, Yavuz, Akif Selim, additional, Panse, Jens, additional, Varkonyi, Judit, additional, Doubek, Michael, additional, Sabato, Vito, additional, Breynaert, Christine, additional, Vucinic, Vladan, additional, Schug, Tanja, additional, Hägglund, Hans, additional, Wortmann, Friederike, additional, Brockow, Knut, additional, Angelova-Fischer, Irena, additional, Belloni Fortina, Anna, additional, Triggiani, Massimo, additional, Reiter, Andreas, additional, Hartmann, Karin, additional, Malcovati, Luca, additional, Gotlib, Jason, additional, Shoumariyeh, Khalid, additional, Niedoszytko, Marek, additional, Arock, Michel, additional, Kluin-Nelemans, Hanneke C., additional, Bonadonna, Patrizia, additional, and Valent, Peter, additional

Published
2021
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44. Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis

Author

Kluin-Nelemans, Hanneke C., Jawhar, Mohamad, Reiter, Andreas, van Anrooij, Bjorn, Gotlib, Jason, Hartmann, Karin, Illerhaus, Anja, Elberink, Hanneke N. G. Oude, Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Mueller, Sabine, Triggiani, Massimo, Parente, Roberta, Schwaab, Juliana, Kundi, Michael, Fortina, Anna Belloni, Caroppo, Francesca, Brockow, Knut, Zink, Alexander, Fuchs, David, Angelova-Fischer, Irena, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hägglund, Hans, Panse, Jens, Simonowski, Anne, Sabato, Vito, Schug, Tanja, Jentzsch, Madlen, Breynaert, Christine, Varkonyi, Judit, Kennedy, Vanessa, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Valent, Peter, Sperr, Wolfgang R., Kluin-Nelemans, Hanneke C., Jawhar, Mohamad, Reiter, Andreas, van Anrooij, Bjorn, Gotlib, Jason, Hartmann, Karin, Illerhaus, Anja, Elberink, Hanneke N. G. Oude, Gorska, Aleksandra, Niedoszytko, Marek, Lange, Magdalena, Scaffidi, Luigi, Zanotti, Roberta, Bonadonna, Patrizia, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Shoumariyeh, Khalid, von Bubnoff, Nikolas, Mueller, Sabine, Triggiani, Massimo, Parente, Roberta, Schwaab, Juliana, Kundi, Michael, Fortina, Anna Belloni, Caroppo, Francesca, Brockow, Knut, Zink, Alexander, Fuchs, David, Angelova-Fischer, Irena, Yavuz, Akif Selim, Doubek, Michael, Mattsson, Mattias, Hägglund, Hans, Panse, Jens, Simonowski, Anne, Sabato, Vito, Schug, Tanja, Jentzsch, Madlen, Breynaert, Christine, Varkonyi, Judit, Kennedy, Vanessa, Hermine, Olivier, Rossignol, Julien, Arock, Michel, Valent, Peter, and Sperr, Wolfgang R.

Abstract

In systemic mastocytosis (SM), the clinical features and survival vary greatly. Patient-related factors determining the outcome in SM are largely unknown. Methods: We examined the impact of sex on the clinical features, progression-free survival (PFS), and overall survival (OS) in 3403 patients with mastocytosis collected in the registry of the European Competence Network on Mastocytosis (ECNM). The impact of cytogenetic and molecular genetic aberrations on sex differences was analyzed in a subset of patients. Results: Of all patients enrolled, 55.3% were females. However, a male predominance was found in a subset of advanced SM (AdvSM) patients, namely SM with an associated hematologic neoplasm (SM-AHN, 70%; p < 0.001). Correspondingly, organomegaly (male: 23% vs. female: 13%, p = 0.007) was more, whereas skin involvement (male: 71% vs. female: 86%, p = 0.001) was less frequent in males. In all patients together, OS (p < 0.0001) was significantly inferior in males, and also within the WHO sub-categories indolent SM, aggressive SM (ASM) and SM-AHN. PFS was significantly (p = 0.0002) worse in males when all patients were grouped together; due to low numbers of events, this significance persisted only in the subcategory smoldering SM. Finally, prognostically relevant cytogenetic abnormalities (10% vs. 5%, p = 0.006) or molecular aberrations (SRSF2/ASXLI/RUNXI profile; 63% vs. 40%, p = 0.003) were more frequently present in males. Conclusions: Male sex has a major impact on clinical features, disease progression, and survival in mastocytosis. Male patients have an inferior survival, which seems related to the fact that they more frequently develop a multi-mutated AdvSM associated with a high-risk molecular background.

Published
2021
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45. Orofacial granulomatosis: Clinical and therapeutic features in an Italian cohort and review of the literature

Author

Galdiero, Maria R., primary, Maio, Filomena, additional, Arcoleo, Francesco, additional, Boni, Elisa, additional, Bonzano, Laura, additional, Brussino, Luisa, additional, Cancian, Mauro, additional, Cremonte, Luigi, additional, Del Giacco, Stefano R., additional, De Paulis, Amato, additional, Detoraki, Aikaterini, additional, Firinu, Davide, additional, Lamacchia, Donatella, additional, Loffredo, Stefania, additional, Nettis, Eustachio, additional, Parente, Roberta, additional, Parronchi, Paola, additional, Pellacani, Giovanni, additional, Petraroli, Angelica, additional, Rolla, Giovanni, additional, Senter, Riccardo, additional, Triggiani, Massimo, additional, Vitiello, Gianfranco, additional, Spadaro, Giuseppe, additional, and Bova, Maria, additional

Published
2021
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46. Familial occurrence of systemic and cutaneous mastocytosis in an adult multicentre series

Author

Tanasi, Ilaria, primary, Bonifacio, Massimiliano, additional, Pizzolato, Miriam, additional, Irene Grifoni, Federica, additional, Sciumè, Mariarita, additional, Elena, Chiara, additional, Benvenuti, Pietro, additional, Mannelli, Francesco, additional, Parente, Roberta, additional, Schena, Donatella, additional, Scaffidi, Luigi, additional, Bonadonna, Patrizia, additional, Papayannidis, Cristina, additional, Rondoni, Michela, additional, Criscuolo, Marianna, additional, Vannucchi, Alessandro M., additional, Triggiani, Massimo, additional, Martinelli, Giovanni, additional, Krampera, Mauro, additional, and Zanotti, Roberta, additional

Published
2021
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47. Vascular Endothelial Growth Factors and Angiopoietins As New Players in Mastocytosis

Author

Marcella, Simone, primary, Petraroli, Angelica, additional, Braile, Mariantonia, additional, Parente, Roberta, additional, Ferrara, Anne Lise, additional, Galdiero, Maria Rosaria, additional, Modestino, Luca, additional, Cristinziano, Leonardo, additional, Rossi, Francesca Wanda, additional, Varricchi, Gilda, additional, Triggiani, Massimo, additional, de Paulis, Amato, additional, Spadaro, Giuseppe, additional, and Loffredo, Stefania, additional

Published
2021
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48. Economic impact of mepolizumab in uncontrolled severe eosinophilic asthma, in real life

Author

Bagnasco, Diego, primary, Povero, Massimiliano, additional, Pradelli, Lorenzo, additional, Brussino, Luisa, additional, Rolla, Giovanni, additional, Caminati, Marco, additional, Menzella, Francesco, additional, Heffler, Enrico, additional, Canonica, Giorgio Walter, additional, Paggiaro, Pierluigi, additional, Senna, Gianenrico, additional, Milanese, Manlio, additional, Lombardi, Carlo, additional, Bucca, Caterina, additional, Manfredi, Andrea, additional, Canevari, Rikki Frank, additional, Passalacqua, Giovanni, additional, Guarnieri, Gabriella, additional, Patella, Vincenzo, additional, Maria Pia, Foschino Barbaro, additional, Carpagnano, Elisiana, additional, Colle, Anna del, additional, Scioscia, Giulia, additional, Gerolamo, Pelaia, additional, Latorre, Manuela, additional, Puggioni, Francesca, additional, Racca, Francesca, additional, Favero, Elisabetta, additional, Iannacone, Sandra, additional, Savi, Eleonora, additional, Montagni, Marcello, additional, Camiciottoli, Gianna, additional, Allegrini, Chiara, additional, Spadaro, Giuseppe, additional, Detoraki, Caterina, additional, Galeone, Carla, additional, Ruggiero, Patrizia, additional, Yacoub, Monna Rita, additional, Berti, Alvise, additional, Colombo, Gisella, additional, Scichilone, Nicola, additional, Durante, Carmen, additional, Costantino, Maria Teresa, additional, Roncallo, Chiara, additional, Braschi, Mariachiara, additional, Blasi, Francesco, additional, D'Adda, Alice, additional, Ridolo, Erminia, additional, Triggiani, Massimo, additional, Parente, Roberta, additional, Maria, D'Amato, additional, Verrillo, Maria Vittoria, additional, Cristina, Zappa Maria, additional, Lilli, Marianna, additional, Crimi, Nunzio, additional, Bonavia, Marco, additional, Corsico, Angelo Guido, additional, Grosso, Amelia, additional, Del Giacco, Stefano, additional, Deidda, Margherita, additional, Ricciardi, Luisa, additional, Isola, Stefania, additional, Cicero, Francesca, additional, Amato, Giuliana, additional, Vita, Federica, additional, Spanevello, Antonio, additional, Pignatti, Patrizia, additional, Cherubino, Francesca, additional, Visca, Dina, additional, Aletti, Eleonora, additional, Massimo Ricciardolo, Fabio Luigi, additional, Anna Carriero, Vitina Maria, additional, Bertolini, Francesca, additional, Santus, Pierachille, additional, Barlassina, Roberta, additional, Airoldi, Andrea, additional, Guida, Giuseppe, additional, Eleonora, Nucera, additional, Aruanno, Arianna, additional, Rizzi, Angela, additional, Caruso, Cristiano, additional, Colantuono, Stefania, additional, Arcolaci, Alessandra, additional, Vianello, Andrea, additional, Bianchi, Fulvia Chieco, additional, Marchi, Maria Rita, additional, Centanni, Stefano, additional, Luraschi, Simone, additional, Ruggeri, Silvia, additional, Rinaldo, Rocco, additional, Parazzini, Elena, additional, Calabrese, Cecilia, additional, Flora, Martina, additional, Cosmi, Lorenzo, additional, Di Pietro, Linda, additional, Maggi, Enrico, additional, Pini, Laura, additional, Macchia, Luigi, additional, Di Bona, Danilo, additional, Richeldi, Luca, additional, Condoluci, Carola, additional, Fuso, Leonello, additional, Bonini, Matteo, additional, Farsi, Alessandro, additional, Carli, Giulia, additional, Montuschi, Paolo, additional, Santini, Giuseppe, additional, Conte, Maria Elisabetta, additional, Turchet, Elisa, additional, Barbetta, Carlo, additional, Mazza, Francesco, additional, D'Alo, Simona, additional, Pucci, Stefano, additional, Caiaffa, Maria Filomena, additional, Minenna, Elena, additional, D'Elia, Luciana, additional, Pasculli, Carlo, additional, Viviano, Vittorio, additional, Tarsia, Paolo, additional, Rolo, Joyce, additional, Di Proietto, Mariacarmela, additional, and Lo Cicero, Salvatore, additional

Published
2021
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49. Cytogenetic and molecular aberrations and worse outcome for male patients in systemic mastocytosis

Author

Kluin-Nelemans, Hanneke C., primary, Jawhar, Mohamad, additional, Reiter, Andreas, additional, van Anrooij, Bjorn, additional, Gotlib, Jason, additional, Hartmann, Karin, additional, Illerhaus, Anja, additional, Oude Elberink, Hanneke N.G., additional, Gorska, Aleksandra, additional, Niedoszytko, Marek, additional, Lange, Magdalena, additional, Scaffidi, Luigi, additional, Zanotti, Roberta, additional, Bonadonna, Patrizia, additional, Perkins, Cecelia, additional, Elena, Chiara, additional, Malcovati, Luca, additional, Shoumariyeh, Khalid, additional, von Bubnoff, Nikolas, additional, Müller, Sabine, additional, Triggiani, Massimo, additional, Parente, Roberta, additional, Schwaab, Juliana, additional, Kundi, Michael, additional, Fortina, Anna Belloni, additional, Caroppo, Francesca, additional, Brockow, Knut, additional, Zink, Alexander, additional, Fuchs, David, additional, Angelova-Fischer, Irena, additional, Yavuz, Akif Selim, additional, Doubek, Michael, additional, Mattsson, Mattias, additional, Hagglund, Hans, additional, Panse, Jens, additional, Simonowski, Anne, additional, Sabato, Vito, additional, Schug, Tanja, additional, Jentzsch, Madlen, additional, Breynaert, Christine, additional, Várkonyi, Judit, additional, Kennedy, Vanessa, additional, Hermine, Olivier, additional, Rossignol, Julien, additional, Arock, Michel, additional, Valent, Peter, additional, and Sperr, Wolfgang R., additional

Published
2021
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50. Clinical features and survival of patients with indolent systemic mastocytosis defined by the updated WHO classification

Author

Trizuljak, Jakub, Sperr, Wolfgang R., Nekvindova, Lucie, Elberink, Hanneke O., Gleixner, Karoline, V, Gorska, Aleksandra, Lange, Magdalena, Hartmann, Karin, Illerhaus, Anja, Bonifacio, Massimiliano, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Fortina, Anna B., Shoumariyeh, Khalid, Jawhar, Mohamad, Zanotti, Roberta, Bonadonna, Patrizia, Caroppo, Francesca, Zink, Alexander, Triggiani, Massimo, Parente, Roberta, von Bubnoff, Nikolas, Yavuz, Akif S., Hägglund, Hans, Mattsson, Mattias, Panse, Jens, Jaekel, Nadja, Kilbertus, Alex, Hermine, Olivier, Arock, Michel, Fuchs, David, Sabato, Vito, Brockow, Knut, Bretterklieber, Agnes, Niedoszytko, Marek, van Anrooij, Bjorn, Reiter, Andreas, Gotlib, Jason, Kluin-Nelemans, Hanneke C., Mayer, Jiri, Doubek, Michael, Valent, Peter, Trizuljak, Jakub, Sperr, Wolfgang R., Nekvindova, Lucie, Elberink, Hanneke O., Gleixner, Karoline, V, Gorska, Aleksandra, Lange, Magdalena, Hartmann, Karin, Illerhaus, Anja, Bonifacio, Massimiliano, Perkins, Cecelia, Elena, Chiara, Malcovati, Luca, Fortina, Anna B., Shoumariyeh, Khalid, Jawhar, Mohamad, Zanotti, Roberta, Bonadonna, Patrizia, Caroppo, Francesca, Zink, Alexander, Triggiani, Massimo, Parente, Roberta, von Bubnoff, Nikolas, Yavuz, Akif S., Hägglund, Hans, Mattsson, Mattias, Panse, Jens, Jaekel, Nadja, Kilbertus, Alex, Hermine, Olivier, Arock, Michel, Fuchs, David, Sabato, Vito, Brockow, Knut, Bretterklieber, Agnes, Niedoszytko, Marek, van Anrooij, Bjorn, Reiter, Andreas, Gotlib, Jason, Kluin-Nelemans, Hanneke C., Mayer, Jiri, Doubek, Michael, and Valent, Peter

Abstract

Background: In indolent systemic mastocytosis (ISM), several risk factors of disease progression have been identified. Previous studies, performed with limited patient numbers, have also shown that the clinical course in ISM is stable and comparable to that of cutaneous mastocytosis (CM). The aim of this project was to compare the prognosis of patients with ISM with that of patients with CM. Methods: We employed a dataset of 1993 patients from the registry of the European Competence Network on Mastocytosis (ECNM) to compare outcomes of ISM and CM. Results: We found that overall survival (OS) is worse in ISM compared to CM. Moreover, in patients with typical ISM, bone marrow mastocytosis (BMM), and smoldering SM (SSM), 4.1% of disease progressions have been observed (4.9% of progressions in typical ISM group, 1.7% in BMM, and 9.4% in SSM). Progressions to advanced SM were observed in 2.9% of these patients. In contrast, six patients with CM (1.7%) converted to ISM and no definitive progression to advanced SM was found. No significant differences in OS and event-free survival (EFS) were found when comparing ISM, BMM, and SSM. Higher risk of both progression and death was significantly associated with male gender, worse performance status, and organomegaly. Conclusion: Our data confirm the clinical impact of the WHO classification that separates ISM from CM and from other SM variants.

Published
2020
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