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1. 170 Celiac disease in selective IgA deficiency patients is associated with T-cell defects

Author

Perez-Andres, Martin, primary, García-Martín, Alberto, additional, Torres, Alba, additional, Arriba, Sonia de, additional, Serrano, Cristina, additional, Sánchez, Ana Isabel, additional, Bastida, Jose María, additional, Aragón, Larraitz, additional, Ines, Sandra Maria, additional, Madruga, Jose Ignacio, additional, Lopes, Susana, additional, Subirá, Dolores, additional, Martins, Catarina, additional, Muñoz, Francisco Javier, additional, Barez, Abelardo, additional, Martin, Alejandro, additional, Hospital, Angie, additional, Arenas, Pedro Pablo, additional, Monzon, David, additional, Requejo, Pedro Mikel, additional, Prieto, Carlos, additional, Jara, Maria, additional, Contreras, Teresa, additional, Sousa, Ana, additional, Marcos, Miguel, additional, Prada, Alvaro Jose, additional, Dongen, Jacques van, additional, and Orfao, Alberto, additional

Published
2024
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2. 174 Age-associated distribution of TH subsets in blood of LOCID vs CVID patients

Author

Perez-Andres, Martin, primary, Torres, Alba, additional, Lopes, Susana, additional, Arriba, Sonia de, additional, Aragón, Larraitz, additional, Serrano, Cristina, additional, Subirá, Dolores, additional, Mercado, Marta Ruiz, additional, Marcos, Miguel, additional, Ines, Sandra Maria, additional, Martins, Catarina, additional, Hurtado, Guillermina, additional, Albarran, Beatriz, additional, Barez, Abelardo, additional, Madruga, Jose Ignacio, additional, Martin, Alejandro, additional, Bastida, Jose María, additional, Davila, Ignacio, additional, Bonroy, Carolien, additional, Neirinck, Jana, additional, Hofmans, Mattias, additional, Arenas, Pedro Pablo, additional, Monzon, David, additional, Requejo, Pedro Mikel, additional, Contreras, Teresa, additional, Jara, Maria, additional, Prieto, Carlos, additional, Prada, Alvaro Jose, additional, Sousa, Ana, additional, Dongen, Jacques Van, additional, and Orfao, Alberto, additional

Published
2024
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4. OA-12 Predictors of unsustained measurable residual disease (MRD) negativity in transplant-eligible multiple myeloma (MM) patients

Author

Guerrero, Camila, primary, Puig, Noemi, additional, Cedena, Teresa, additional, Calasanz, Maria-Jose, additional, Gutierrez, Norma, additional, Fernandez-Guijarro, Manuela, additional, Oriol, Albert, additional, Rios, Rafael, additional, Garcia, Miguel Teodoro Hernandez, additional, Martinez-Martinez, Rafael, additional, Bargay, Joan, additional, de la Fuente, Felipe de Arriba, additional, Palomera, Luis, additional, Gonzalez-Rodriguez, Ana Pilar, additional, Gonzalez-Perez, Marta-Sonia, additional, Orfao, Alberto, additional, Mateos, Maria-Victoria, additional, Martinez-Lopez, Joaquin, additional, Rosinol, Laura, additional, Bladé, Joan, additional, Palacios, Juan José Lahuerta, additional, San-Miguel, Jesús, additional, and Paiva, Bruno, additional

Published
2023
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6. The Normal Range of Baseline Tryptase Should Be 1 to 15 ng/mL and Covers Healthy Individuals With HαT

Author

Valent, Peter, primary, Hoermann, Gregor, additional, Bonadonna, Patrizia, additional, Hartmann, Karin, additional, Sperr, Wolfgang R., additional, Broesby-Olsen, Sigurd, additional, Brockow, Knut, additional, Niedoszytko, Marek, additional, Hermine, Olivier, additional, Chantran, Yannick, additional, Butterfield, Joseph H., additional, Greiner, Georg, additional, Carter, Melody C., additional, Sabato, Vito, additional, Radia, Deepti H., additional, Siebenhaar, Frank, additional, Triggiani, Massimo, additional, Gülen, Theo, additional, Alvarez-Twose, Ivan, additional, Staudinger, Thomas, additional, Traby, Ludwig, additional, Sotlar, Karl, additional, Reiter, Andreas, additional, Horny, Hans-Peter, additional, Orfao, Alberto, additional, Galli, Stephen J., additional, Schwartz, Lawrence B., additional, Lyons, Jonathan J., additional, Gotlib, Jason, additional, Metcalfe, Dean D., additional, Arock, Michel, additional, and Akin, Cem, additional

Published
2023
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7. Cytochrome P450-soluble epoxide hydrolase derived linoleic acid oxylipins and cognitive performance in type 2 diabetes

Author

Anita, Natasha Z., primary, Kwan, Felicia, additional, Ryoo, Si Won, additional, Major-Orfao, Chelsi, additional, Lin, William Z., additional, Noor, Shiropa, additional, Lanctôt, Krista L., additional, Herrmann, Nathan, additional, Oh, Paul I., additional, Shah, Baiju R., additional, Gilbert, Jeremy, additional, Assal, Angela, additional, Halperin, Ilana J., additional, Taha, Ameer Y., additional, and Swardfager, Walter, additional

Published
2023
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8. Immunotherapy: HIGH INCIDENCE OF CYTOPENIAS AFTER COMMERCIAL CAR-T CELL THERAPY IN A REAL-WORLD SETTING: UTILITY OF THE CAR-HEMATOTOX INDEX

Author

A. Navarro-Bailon, A. Martin, V. Martin-Mendez, L. Prieto, E. Perez-Lopez, A. Cabero, M. Garcia-Blazquez, M. Fernandez-Crespo, S. Muntión, S. Preciado, M. Gomez-Redondo, M. Alaña, N. Albala, M. Lopez-Parra, P. Tamayo, E. Villaron, A. Yeguas, M. Alcoceba, A. Orfao, N. Gutierrez, L. Lopez-Corral, and F. Sanchez-Guijo

Subjects
Cancer Research, Transplantation, Oncology, Immunology, Immunology and Allergy, Cell Biology, Genetics (clinical)
Published
2023

9. European Competence Network on Mastocytosis (ECNM): 20-Year Jubilee, Updates, and Future Perspectives

Author

Valent, Peter, primary, Hartmann, Karin, additional, Bonadonna, Patrizia, additional, Sperr, Wolfgang R., additional, Niedoszytko, Marek, additional, Hermine, Olivier, additional, Kluin-Nelemans, Hanneke C., additional, Sotlar, Karl, additional, Hoermann, Gregor, additional, Nedoszytko, Boguslaw, additional, Broesby-Olsen, Sigurd, additional, Zanotti, Roberta, additional, Lange, Magdalena, additional, Doubek, Michael, additional, Brockow, Knut, additional, Alvarez-Twose, Ivan, additional, Varkonyi, Judit, additional, Yavuz, Selim, additional, Nilsson, Gunnar, additional, Radia, Deepti, additional, Grattan, Clive, additional, Schwaab, Juliana, additional, Gülen, Theo, additional, Oude Elberink, Hanneke N.G., additional, Hägglund, Hans, additional, Siebenhaar, Frank, additional, Hadzijusufovic, Emir, additional, Sabato, Vito, additional, Mayer, Jiri, additional, Reiter, Andreas, additional, Orfao, Alberto, additional, Horny, Hans-Peter, additional, Triggiani, Massimo, additional, and Arock, Michel, additional

Published
2023
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10. Immunotherapy: HIGH INCIDENCE OF CYTOPENIAS AFTER COMMERCIAL CAR-T CELL THERAPY IN A REAL-WORLD SETTING: UTILITY OF THE CAR-HEMATOTOX INDEX

Author

Navarro-Bailon, A., primary, Martin, A., additional, Martin-Mendez, V., additional, Prieto, L., additional, Perez-Lopez, E., additional, Cabero, A., additional, Garcia-Blazquez, M., additional, Fernandez-Crespo, M., additional, Muntión, S., additional, Preciado, S., additional, Gomez-Redondo, M., additional, Alaña, M., additional, Albala, N., additional, Lopez-Parra, M., additional, Tamayo, P., additional, Villaron, E., additional, Yeguas, A., additional, Alcoceba, M., additional, Orfao, A., additional, Gutierrez, N., additional, Lopez-Corral, L., additional, and Sanchez-Guijo, F., additional

Published
2023
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11. Afección pericárdica y miocárdica tras infección por SARS-CoV-2: estudio descriptivo transversal en trabajadores sanitarios

Author

Rocío Eiros, Manuel Barreiro-Pérez, Ana Martín-García, Julia Almeida, Eduardo Villacorta, Alba Pérez-Pons, Soraya Merchán, Alba Torres-Valle, Clara Sánchez-Pablo, David González-Calle, Oihane Pérez-Escurza, Inés Toranzo, Elena Díaz-Peláez, Blanca Fuentes-Herrero, Laura Macías-Álvarez, Guillermo Oliva-Ariza, Quentin Lecrevisse, Rafael Fluxa, José L. Bravo-Grande, Alberto Orfao, Pedro L. Sánchez, Instituto de Salud Carlos III, European Commission, Ministerio de Ciencia, Innovación y Universidades (España), and Junta de Castilla y León

Subjects
Cardiac magnetic resonance, SARS-CoV-2, Immune cells, COVID-19, Células inmunes, Daño cardiaco, Respuesta inmune, Cardiac injury, Serología, Myocarditis, Resonancia magnética cardiaca, Serology, Healthcare worker, Pericarditis, Trabajador sanitario, Miocarditis, Immune response, Cardiology and Cardiovascular Medicine
Abstract

[EN] Introduction and objectives The cardiac sequelae of SARS-CoV-2 infection are still poorly documented. We conducted a cross-sectional study in healthcare workers to report evidence of pericardial and myocardial involvement after SARS-CoV-2 infection. Methods We studied 139 healthcare workers with confirmed past SARS-CoV-2 infection. Participants underwent clinical assessment, electrocardiography, and laboratory tests, including immune cell profiling and cardiac magnetic resonance (CMR). Clinically suspected pericarditis was diagnosed when classic criteria were present and clinically suspected myocarditis was based on the combination of at least 2 CMR criteria. Results Median age was 52 (41-57) years, 71.9% were women, and 16.5% were previously hospitalized for COVID-19 pneumonia. On examination (10.4 [9.3-11.0] weeks after infection-like symptoms), participants showed hemodynamic stability. Chest pain, dyspnea or palpitations were present in 41.7% participants, electrocardiographic abnormalities in 49.6%, NT-proBNP elevation in 7.9%, troponin in 0.7%, and CMR abnormalities in 60.4%. A total of 30.9% participants met criteria for either pericarditis and/or myocarditis: isolated pericarditis was diagnosed in 5.8%, myopericarditis in 7.9%, and isolated myocarditis in 17.3%. Most participants (73.2%) showed altered immune cell counts in blood, particularly decreased eosinophil (27.3%; P < .001) and increased cytotoxic T cell numbers (17.3%; P < .001). Clinically suspected pericarditis was associated (P < .005) with particularly elevated cytotoxic T cells and decreased eosinophil counts, while participants diagnosed with clinically suspected myopericarditis or myocarditis had lower (P < .05) neutrophil counts, natural killer-cells, and plasma cells. Conclusions Pericardial and myocardial involvement with clinical stability are frequent after SARS-CoV-2 infection and are associated with specific immune cell profiles., [ES] Introducción y objetivos Las secuelas cardiacas tras la infección por SARS-CoV-2 todavía están poco documentadas. Se realizó un estudio transversal en trabajadores sanitarios para estudiar la prevalencia de afección pericárdica y miocárdica tras la infección por SARS-CoV-2. Métodos Se estudió a 139 trabajadores sanitarios con infección previa confirmada por SARS-CoV-2. Los participantes se sometieron a evaluación clínica, electrocardiograma, laboratorio, incluido el perfil de células inmunitarias, y resonancia magnética cardiaca (RMC). El diagnóstico clínico de pericarditis se realizó ante la presencia de los criterios clásicos y el diagnóstico clínico de miocarditis ante la presencia de al menos 2 criterios de RMC. Resultados La mediana de edad fue de 52 (41–57) años, el 71,9% eran mujeres, y el 16.5% había sido hospitalizado previamente por neumonía por COVID-19. En la evaluación (10,4 [9,3–11,0] semanas después de los síntomas de infección), todos los participantes presentaban estabilidad hemodinámica. El 41,7% presentaba dolor torácico, disnea o palpitaciones; el 49,6%, alteraciones electrocardiográficas; el 7,9%, elevación de NT-proBNP; el 0,7%, elevación de troponina; y el 60,4%, alteraciones en la RMC. Un total de 30,9% de participantes cumplieron los criterios clínicos establecidos de pericarditis o miocarditis: pericarditis aislada en el 5,8%, miopericarditis en el 7,9% y miocarditis aislada en el 17,3%. La mayoría de los participantes (73,2%) mostraron recuentos de células inmunitarias alterados en sangre; en particular diminución de eosinófilos (27,3%; p < 0,001) y aumento del número de células T citotóxicas (17,3%; p < 0,001). La sospecha clínica de pericarditis se asoció (p < 0,005) particularmente con un elevado número de células T citotóxicas y recuento de eosinófilos disminuidos; mientras que los participantes con sospecha clínica de miopericarditis o miocarditis tenían recuentos de neutrófilos, células natural killer y células plasmáticas más bajos (p < 0,05). Conclusiones La afección pericárdica y miocárdica con estabilidad hemodinámica es frecuente después de la infección por SARS-CoV-2 y se asocia con perfiles de células inmunitarias específicas., This study was supported by CIBERCV (CB16/11/00374), CIBERONC (CB16/12/00400) and the COV20/00386 grant from the Instituto de Salud Carlos III and FEDER, Ministerio de Ciencia e Innovación, Madrid, Spain, and by GRS COVID 26/A/20 from the Gerencia Regional de Salud, Junta de Castilla y León, Spain.

Published
2022

12. Standards of Genetic Testing in the Diagnosis and Prognostication of Systemic Mastocytosis in 2022: Recommendations of the EU-US Cooperative Group

Author

Gregor Hoermann, Karl Sotlar, Mohamad Jawhar, Thomas Kristensen, Guillaume Bachelot, Boguslaw Nedoszytko, Melody C. Carter, Hans-Peter Horny, Patrizia Bonadonna, Wolfgang R. Sperr, Karin Hartmann, Knut Brockow, Jonathan J. Lyons, Hanneke C. Kluin-Nelemans, Olivier Hermine, Cem Akin, Sigurd Broesby-Olsen, Massimo Triggiani, Joseph H. Butterfield, Juliana Schwaab, Andreas Reiter, Jason Gotlib, Dean D. Metcalfe, Tracy I. George, Alberto Orfao, Peter Valent, Michel Arock, National Institute of Allergy and Infectious Diseases (US), National Institutes of Health (US), and Austrian Science Fund

Subjects
allele burden, diagnosis, KIT mutations, Real-Time Polymerase Chain Reaction, Mast cell, Proto-Oncogene Proteins c-kit, Mastocytosis, Systemic, mastocytosis, next-generation sequencing, prognosis, Mutation, Humans, Immunology and Allergy, Genetic Testing, Mast Cells, Mastocytosis
Abstract

Mastocytosis comprises rare heterogeneous diseases characterized by an increased accumulation of abnormal mast cells in various organs/tissues. The pathogenesis of mastocytosis is strongly linked to the presence of KIT-activating mutations. In systemic mastocytosis (SM), the most frequent mutation encountered is KIT p.D816V, whose presence constitutes one of the minor diagnostic criteria. Different techniques are used to search and quantify the KIT p.D816V mutant; however, allele-specific quantitative PCR and droplet digital PCR are today the most sensitive. The analysis of the KIT p.D816V allele burden has undeniable interest for diagnostic, prognostic, and therapeutic monitoring. The analysis of non–mast cell hematological compartments in SM is similarly important because KIT p.D816V multilineage involvement is associated with a worse prognosis. In addition, in advanced forms of SM, mutations in genes other than KIT are frequently identified and affect negatively disease outcome and response to therapy. Thus, combined quantitative and sensitive analysis of KIT mutations and next-generation sequencing of other recurrently involved myeloid genes make it possible to better characterize the extent of the affected cellular compartments and additional molecular aberrations, providing a more detailed overview of the complex mutational landscape of SM, in relation with the clinical heterogeneity of the disease. In this article, we report the latest recommendations of the EU-US Cooperative Group presented in September 2020 in Vienna during an international working conference, on the techniques we consider standard to detect and quantify the KIT p.D816V mutant in SM and additional myeloid mutations found in SM subtypes., D.D.M., J.J.L., and M.C.C. were supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health. P.V. was supported by the Austrian Science Fund (FWF) (grant nos. F4704-B20 and P32470-B).

Published
2022

13. Global Classification of Mast Cell Activation Disorders: An ICD-10-CM–Adjusted Proposal of the ECNM-AIM Consortium

Author

Peter Valent, Karin Hartmann, Patrizia Bonadonna, Theo Gülen, Knut Brockow, Ivan Alvarez-Twose, Olivier Hermine, Marek Niedoszytko, Melody C. Carter, Gregor Hoermann, Joseph H. Butterfield, Jonathan J. Lyons, Wolfgang R. Sperr, Georg Greiner, Karl Sotlar, Hanneke C. Kluin-Nelemans, Juliana Schwaab, Magdalena Lange, Tracy I. George, Frank Siebenhaar, Sigurd Broesby-Olsen, Mohamad Jawhar, Boguslaw Nedoszytko, Mariana Castells, Alberto Orfao, Jason Gotlib, Andreas Reiter, Hans-Peter Horny, Massimo Triggiani, Michel Arock, Dean D. Metcalfe, Cem Akin, Lindbergh Foundation, Stockholm County Council, National Institute of Allergy and Infectious Diseases (US), Austrian Science Fund, and Publica

Subjects
Diagnostic criteria, HαT, Mast cell activation, Mastocytosis, MCAS, Hypersensitivity, Immediate, Immunoglobulin E, Mast Cell Activation Disorders, International Classification of Diseases, Humans, Immunology and Allergy, Tryptases, Mast Cells
Abstract

Mast cell activation (MCA) is common and occurs in a number of pathologic conditions, including IgE-dependent and independent allergic reactions, atopic disorders, autoimmune processes, and mastocytosis. In a subset of patients, no underlying disease and no known trigger of MCA are found. When the symptoms are severe, systemic, and recurrent, and accompanied by a diagnostic increase in the serum tryptase level or other mast cell mediators, an MCA syndrome (MCAS) may be diagnosed. In these patients, the symptoms typically respond to drugs suppressing MCA, mediator production in mast cells, or mediator effects. In each case, diagnostic consensus criteria must be fulfilled to diagnose MCAS. In other patients, MCA may be local, less severe, or less acute, or may be suspected but not confirmed, so that the diagnostic criteria of MCAS are not fulfilled. In these patients, it may be difficult to prove MCA, for example, by measuring multiple mast cell mediators or basophil activation, the latter as a surrogate of IgE-dependent hypersensitivity. However, validated diagnostic criteria for implicating suspected MCA behind such conditions are lacking, even if some of these conditions have recently been assigned to an International Classification of Diseases-10-Clinical Modification code (ICD-10-CM). In this article, we discuss diagnostic features and criteria and propose a ICD-10-CM–adjusted classification for disorders associated with MCA, herein referred to as MCA disorders (MCADs), with special emphasis on the delineation between confirmed MCAS, MCAD not fulfilling MCAS criteria, and suspected MCAD that is not present. In addition, we discuss the discrimination between overt MCAD and predisposing conditions, such as atopic states, mastocytosis, and hereditary alpha tryptasemia., T.G. was supported by grants from the Konsul TH C Bergh Foundation and the Stockholm County Council Research Funds (ALF), Sweden. M.C.C., J.J.L, and D.D.M. were supported by the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIH). The content is solely the responsibility of the authors and does not represent the official views of the NIH. P.V. was supported by the Austrian Science Fund (FWF; projects P32470-B and F4704-B20).

Published
2022

14. Refined Treatment Response Criteria for Indolent Systemic Mastocytosis Proposed by the ECNM-AIM Consortium

Author

Pyatilova, Polina, Akin, Cem, Álvarez-Twose, Iván, Arock, Michel, Bonadonna, Patrizia, Brockow, Knut, Butterfield, Joseph H., Broesby-Olsen, Sigurd, Carter, Melody C., Castells, Mariana, George, Tracy I., Gotlib, Jason, Greiner, Georg, Gulen, Theo, Hartmann, Karin, Hermine, Olivier, Horny, Hans-Peter, Jawhar, Mohamad, Lange, Magdalena, Lyons, Jonathan J., Maurer, Marcus, Metcalfe, Dean D., Nedoszytko, Boguslaw, Niedoszytko, Marek, Orfao, Alberto, Reiter, Andreas, Schwaab, Juliana, Sotlar, Karl, Sperr, Wolfgang R., Triggiani, Massimo, Valent, Peter, Siebenhaar, Frank, National Institute of Allergy and Infectious Diseases (US), National Institutes of Health (US), Medical University of Gdańsk, Austrian Science Fund, and Publica

Subjects
Mastocytosis, Systemic, Mast cell activation, Mast cells, Mastocytosis, Response criteria, Signs, Symptoms, Quality of Life, Humans, Immunology and Allergy, Tryptases
Abstract

Indolent systemic mastocytosis (ISM) has a favorable prognosis and normal life expectancy. However, many patients suffer from mast cell (MC) mediator-related symptoms, which significantly affect quality of life (QoL). Cutaneous, gastrointestinal, and neurological complaints, musculoskeletal pain, and the presence of skin lesions, anaphylaxis, and osteoporosis are the main symptoms and signs in ISM and must be assessed in all patients before and during treatment. Validated mastocytosis-specific patient-reported outcome measures (PROMs) should be used for this purpose. Serum tryptase and KIT D816V allele burden are recommended as secondary outcome parameters, noting that they do not reflect the severity of signs, symptoms, and related QoL impairment, but indirectly express MC burden. Changes from baseline of 90%, 60%, and 30% indicate complete response >90%, major response 60% to 90%, partial response 30% to 60%, and no response, M. C. Carter, J. J. Lyons, and D. D. Metcalfe were supported by the Division of Intramural Research, National Institutes of Allergic and Infectious Diseases, and National Institutes of Health. M. Niedoszytko was supported by the Medical University of Gdansk grant 02-0141/07/231. P. Valent was supported by the Austrian Science Fund (FWF) grant # P32470-B.

Published
2022

15. European Competence Network on Mastocytosis (ECNM): 20-Year Jubilee, Updates, and Future Perspectives

Author

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, and Michel Arock

Subjects
ECNM, Mast cells, MCAS, Immunology and Allergy, Mastocytosis, Mast cell activation disorders
Abstract

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. The ECNM also organized annual meetings and several working conferences, thereby supporting the development of the World Health Organization classification between 2002 and 2022. In addition, the ECNM established a robust and rapidly expanding patient registry and supported the development of new prognostic scoring systems and new treatment approaches. In all projects, ECNM representatives collaborated closely with their U.S. colleagues, various patient organizations, and other scientific networks. Finally, ECNM members have started several collaborations with industrial partners, leading to the preclinical development and clinical testing of KIT-targeting drugs in systemic mastocytosis, and some of these drugs received licensing approval in recent years. All these networking activities and collaborations have strengthened the ECNM and supported our efforts to increase awareness of MC disorders and to improve diagnosis, prognostication, and therapy in patients.

Published
2023

16. Poster: ALL-268 Genetic Classification of B-Cell Precursor Adult Acute Lymphoblastic Leukemia Patients Enrolled in LAL19 Trial from the Pethema Group: Response to Treatment and Survival

Author

Ribera, Jordi, primary, Granada, Isabel, additional, González, Teresa, additional, Morgades, Mireia, additional, Sánchez, Ricardo, additional, Such, Esperanza, additional, Barrena, Susana, additional, Ciudad, Juana, additional, Soriano, Beatriz, additional, Benito, Rocío, additional, Avetisyan, Gayane, additional, Lumbreras, Eva, additional, Miguel, Cristina, additional, Santos, Sandra, additional, Zamora, Lurdes, additional, Mallo, Mar, additional, Genescà, Eulàlia, additional, González, Celia, additional, Lopes, Thaysa, additional, Hernández-Rivas, Jesús-María, additional, Orfao, Alberto, additional, and Ribera, Josep Maria, additional

Published
2022
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17. ALL-268 Genetic Classification of B-Cell Precursor Adult Acute Lymphoblastic Leukemia Patients Enrolled in LAL19 Trial from the Pethema Group: Response to Treatment and Survival

Author

Ribera, Jordi, primary, Granada, Isabel, additional, González, Teresa, additional, Morgades, Mireia, additional, Sánchez, Ricardo, additional, Such, Esperanza, additional, Barrena, Susana, additional, Ciudad, Juana, additional, Soriano, Beatriz, additional, Benito, Rocío, additional, Avetisyan, Gayane, additional, Lumbreras, Eva, additional, Miguel, Cristina, additional, Santos, Sandra, additional, Zamora, Lurdes, additional, Mallo, Mar, additional, Genescà, Eulàlia, additional, González, Celia, additional, Lopes, Thaysa, additional, Hernández-Rivas, Jesús-María, additional, Orfao, Alberto, additional, and Ribera, Josep Maria, additional

Published
2022
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18. Pathogenic and diagnostic relevance of KIT in primary mast cell activation disorders

Author

Iván Álvarez-Twose, Andrés C. García-Montero, Javier I. Muñoz-González, Alberto Orfao, Instituto de Salud Carlos III, European Commission, Centro de Investigación Biomédica en Red Cáncer (España), and Fundación Española de Mastocitosis

Subjects
Pulmonary and Respiratory Medicine, Immunology, Immunoglobulin E, Pathogenesis, Mastocytosis, Systemic, Protein Domains, medicine, Humans, Point Mutation, Immunology and Allergy, Mast Cells, Systemic mastocytosis, Frameshift Mutation, Anaphylaxis, biology, business.industry, Cutaneous Mastocytosis, medicine.disease, Mast cell, Proto-Oncogene Proteins c-kit, medicine.anatomical_structure, Monoclonal, biology.protein, Tryptases, Bone marrow, Inflammation Mediators, business
Abstract

[Objective]: Mast cell (MC) activation (MCA) defines the mechanism by which certain patients have symptoms owing to the effect of a wide range of mediators released from MCs upon their activation, when triggered by different stimuli. When these symptoms are severe and recurrent, the diagnosis of MCA syndrome (MCAS) might be considered. Here, we review the relevant aspects related to the pathogenesis of MCAS, with special emphasis on the prevalence and diagnostic relevance of KIT mutations., [Data Sources]: PubMed was searched between 1980 and 2021 using the following terms: mast cell activation syndromes, mast cell activation, anaphylaxis, KIT mutations, KIT D816V, indolent systemic mastocytosis, bone marrow mastocytosis, cutaneous mastocytosis, IgE anaphylaxis, and idiopathic anaphylaxis., [Study Selections]: Only articles published in English were selected based on their relevance to MCAS or severe and recurrent anaphylaxis., [Results]: MCAS can be classified as clonal MCAS and nonclonal MCAS depending on the presence vs absence of an underlying KIT mutation (mostly KIT D816V), respectively. In contrast to clonal MCAS in which MCA is associated with a primary MC disorder (ie, primary MCAS) such as mastocytosis or monoclonal MCAS, nonclonal MCAS can be secondary to known or unidentified triggers (ie, secondary and idiopathic MCAS, respectively)., [Conclusion]: The clinical heterogeneity and complexity of the molecular assays needed for the study of patients with MCAS might lead to misdiagnosis, particularly when patients are evaluated at nonspecialized centers. Thus, referral of patients having clinical manifestations suggestive of MCAS to reference centers on mastocytosis and MC diseases is strongly recommended., This work was supported by grants from the Instituto de Salud Carlos III (Madrid, Spain), Fondos Europeos de Desarrollo Regional (reference numbers: PI19/01166 and Centro de Investigación Biomédica en Red de Cáncer, CB16/12/00400), Fundación Española de Mastocitosis (Madrid, Spain; reference number: FEM2021-SAM), and Asociación Española de Pacientes con Mastocitosis y otras Enfermedades Relacionadas (Madrid, Spain; reference number: AEDM2019-SAM).

Published
2021

19. Afección pericárdica y miocárdica tras infección por SARS-CoV-2: estudio descriptivo transversal en trabajadores sanitarios

Author

Eiros, Rocío, primary, Barreiro-Pérez, Manuel, additional, Martín-García, Ana, additional, Almeida, Julia, additional, Villacorta, Eduardo, additional, Pérez-Pons, Alba, additional, Merchán, Soraya, additional, Torres-Valle, Alba, additional, Sánchez-Pablo, Clara, additional, González-Calle, David, additional, Pérez-Escurza, Oihane, additional, Toranzo, Inés, additional, Díaz-Peláez, Elena, additional, Fuentes-Herrero, Blanca, additional, Macías-Álvarez, Laura, additional, Oliva-Ariza, Guillermo, additional, Lecrevisse, Quentin, additional, Fluxa, Rafael, additional, Bravo-Grande, José L., additional, Orfao, Alberto, additional, and Sánchez, Pedro L., additional

Published
2022
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20. Pericardial and myocardial involvement after SARS-CoV-2 infection: a cross-sectional descriptive study in healthcare workers

Author

Eiros, Rocío, primary, Barreiro-Pérez, Manuel, additional, Martín-García, Ana, additional, Almeida, Julia, additional, Villacorta, Eduardo, additional, Pérez-Pons, Alba, additional, Merchán, Soraya, additional, Torres-Valle, Alba, additional, Sánchez-Pablo, Clara, additional, González-Calle, David, additional, Pérez-Escurza, Oihane, additional, Toranzo, Inés, additional, Díaz-Peláez, Elena, additional, Fuentes-Herrero, Blanca, additional, Macías-Álvarez, Laura, additional, Oliva-Ariza, Guillermo, additional, Lecrevisse, Quentin, additional, Fluxa, Rafael, additional, Bravo-Grande, José L., additional, Orfao, Alberto, additional, and Sánchez, Pedro L., additional

Published
2022
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21. Standards of Genetic Testing in the Diagnosis and Prognostication of Systemic Mastocytosis in 2022: Recommendations of the EU-US Cooperative Group

Author

Hoermann, Gregor, primary, Sotlar, Karl, additional, Jawhar, Mohamad, additional, Kristensen, Thomas, additional, Bachelot, Guillaume, additional, Nedoszytko, Boguslaw, additional, Carter, Melody C., additional, Horny, Hans-Peter, additional, Bonadonna, Patrizia, additional, Sperr, Wolfgang R., additional, Hartmann, Karin, additional, Brockow, Knut, additional, Lyons, Jonathan J., additional, Kluin-Nelemans, Hanneke C., additional, Hermine, Olivier, additional, Akin, Cem, additional, Broesby-Olsen, Sigurd, additional, Triggiani, Massimo, additional, Butterfield, Joseph H., additional, Schwaab, Juliana, additional, Reiter, Andreas, additional, Gotlib, Jason, additional, Metcalfe, Dean D., additional, George, Tracy I., additional, Orfao, Alberto, additional, Valent, Peter, additional, and Arock, Michel, additional

Published
2022
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22. Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User’s Guide for Daily Clinical Practice

Author

Valent, Peter, primary, Hartmann, Karin, additional, Schwaab, Juliana, additional, Alvarez-Twose, Ivan, additional, Brockow, Knut, additional, Bonadonna, Patrizia, additional, Hermine, Olivier, additional, Niedoszytko, Marek, additional, Carter, Melody C., additional, Hoermann, Gregor, additional, Sperr, Wolfgang R., additional, Butterfield, Joseph H., additional, Ustun, Celalettin, additional, Zanotti, Roberta, additional, Radia, Deepti H., additional, Castells, Mariana, additional, Triggiani, Massimo, additional, Schwartz, Lawrence B., additional, Orfao, Alberto, additional, George, Tracy I., additional, Sotlar, Karl, additional, Gotlib, Jason, additional, Reiter, Andreas, additional, Horny, Hans-Peter, additional, Arock, Michel, additional, Akin, Cem, additional, and Metcalfe, Dean D., additional

Published
2022
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23. Global Classification of Mast Cell Activation Disorders: An ICD-10-CM–Adjusted Proposal of the ECNM-AIM Consortium

Author

Valent, Peter, primary, Hartmann, Karin, additional, Bonadonna, Patrizia, additional, Gülen, Theo, additional, Brockow, Knut, additional, Alvarez-Twose, Ivan, additional, Hermine, Olivier, additional, Niedoszytko, Marek, additional, Carter, Melody C., additional, Hoermann, Gregor, additional, Butterfield, Joseph H., additional, Lyons, Jonathan J., additional, Sperr, Wolfgang R., additional, Greiner, Georg, additional, Sotlar, Karl, additional, Kluin-Nelemans, Hanneke C., additional, Schwaab, Juliana, additional, Lange, Magdalena, additional, George, Tracy I., additional, Siebenhaar, Frank, additional, Broesby-Olsen, Sigurd, additional, Jawhar, Mohamad, additional, Nedoszytko, Boguslaw, additional, Castells, Mariana, additional, Orfao, Alberto, additional, Gotlib, Jason, additional, Reiter, Andreas, additional, Horny, Hans-Peter, additional, Triggiani, Massimo, additional, Arock, Michel, additional, Metcalfe, Dean D., additional, and Akin, Cem, additional

Published
2022
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24. Proposed European Competence Network on Mastocytosis—American Initiative in Mast Cell Diseases (ECNM-AIM) Response Criteria in Advanced Systemic Mastocytosis

Author

Gotlib, Jason, primary, Schwaab, Juliana, additional, Shomali, William, additional, George, Tracy I., additional, Radia, Deepti H., additional, Castells, Mariana, additional, Carter, Melody C., additional, Hartmann, Karin, additional, Álvarez-Twose, Ivan, additional, Brockow, Knut, additional, Bonadonna, Patrizia, additional, Hermine, Olivier, additional, Niedoszytko, Marek, additional, Hoermann, Gregor, additional, Sperr, Wolfgang R., additional, Elberink, Hanneke Oude, additional, Siebenhaar, Frank, additional, Butterfield, Joseph H., additional, Ustun, Celalettin, additional, Zanotti, Roberta, additional, Triggiani, Massimo, additional, Schwartz, Lawrence B., additional, Lyons, Jonathan J., additional, Orfao, Alberto, additional, Sotlar, Karl, additional, Horny, Hans-Peter, additional, Arock, Michel, additional, Metcalfe, Dean D., additional, Akin, Cem, additional, Lübke, Johannes, additional, Valent, Peter, additional, and Reiter, Andreas, additional

Published
2022
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25. Mast Cell Diseases in Practice and Research: Issues and Perspectives Raised by Patients and Their Recommendations to the Scientific Community and Beyond

Author

Jennings, Susan V., primary, Finnerty, Celeste C., additional, Hobart, Jessica S., additional, Martín-Martínez, Mercedes, additional, Sinclair, Kristin A., additional, Slee, Valerie M., additional, Agopian, Julie, additional, Akin, Cem, additional, Álvarez-Twose, Ivan, additional, Bonadonna, Patrizia, additional, Bowman, Angela S., additional, Brockow, Knut, additional, Bumbea, Horia, additional, de Haro, Claudia, additional, Fok, Jie Shen, additional, Hartmann, Karin, additional, Hegmann, Nicole, additional, Hermine, Olivier, additional, Kalisiak, Monika, additional, Katelaris, Constance H., additional, Kurz, Jacqueline, additional, Marcis, Patrizia, additional, Mayne, David, additional, Mendoza, David, additional, Moussy, Alain, additional, Mudretzkyj, Genija, additional, Vaia, Nicoleta Nidelea, additional, Niedoszytko, Marek, additional, Elberink, Hanneke Oude, additional, Orfao, Alberto, additional, Radia, Deepti H., additional, Rosenmeier, Sophie, additional, Ribada, Eugenia, additional, Schinhofen, Waltraud, additional, Schwaab, Juliana, additional, Siebenhaar, Frank, additional, Triggiani, Massimo, additional, Tripodo, Giuseppe, additional, Velazquez, Rocio, additional, Wielink, Yvon, additional, Wimazal, Friedrich, additional, Yigit, Timo, additional, Zubrinich, Celia, additional, and Valent, Peter, additional

Published
2022
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26. Standards of Pathology in the Diagnosis of Systemic Mastocytosis: Recommendations of the EU-US Cooperative Group

Author

Sotlar, Karl, primary, George, Tracy I., additional, Kluin, Philip, additional, Reiter, Andreas, additional, Schwaab, Juliana, additional, Panse, Jens, additional, Brockow, Knut, additional, Hartmann, Karin, additional, Sperr, Wolfgang R., additional, Kristensen, Thomas, additional, Nedoszytko, Boguslaw, additional, Carter, Melody, additional, Bonadonna, Patrizia, additional, Lyons, Jonathan J., additional, Kluin-Nelemans, Hanneke C., additional, Hermine, Olivier, additional, Akin, Cem, additional, Broesby-Olsen, Sigurd, additional, Hoermann, Gregor, additional, Triggiani, Massimo, additional, Butterfield, Joseph H., additional, Jawhar, Mohamad, additional, Gotlib, Jason, additional, Metcalfe, Dean D., additional, Orfao, Alberto, additional, Arock, Michel, additional, Valent, Peter, additional, and Horny, Hans-Peter, additional

Published
2022
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28. Proposed global prognostic score for systemic mastocytosis: a retrospective prognostic modelling study

Author

Blanca Xicoy, Peter Valent, Juliana Schwaab, Khalid Shoumariyeh, Enrique Colado, Francesco Olivieri, Annette Schmitt-Graeff, Javier I. Muñoz-González, Andrés C. García-Montero, Georg Greiner, Iván Álvarez-Twose, Carlos Fernandez-Gimenez, Andreas Reiter, Jason Gotlib, María Jara-Acevedo, Ana Gabriela Henriques, Roberta Zanotti, Andrea Mayado, Alba Pérez-Pons, Cecelia Perkins, Wolfgang R. Sperr, Irene Luna, Mohamad Jawhar, Elvira Mora-Casterá, Maria-Helena Bañas, Ilaria Tanasi, Patrizia Bonadonna, Guillermo Martín-Sánchez, Laura Sánchez-Muñoz, Georgina Gener-Ricós, Amanda Nuñez-García, Manuel Jurado-Chacón, Leonor Senent, Justus Duyster, Carolina Caldas, Alberto Orfao, Instituto de Salud Carlos III, European Commission, The Mastocytosis Society (US), Ministerio de Sanidad (España), Junta de Castilla y León, Charles and Ann Johnson Foundation, and Austrian Science Fund

Subjects
Adult, Male, Oncology, MIDOSTAURIN, medicine.medical_specialty, DIVERSITY, Kaplan-Meier Estimate, Polymorphism, Single Nucleotide, CLASSIFICATION, Prognostic score, Hemoglobins, 03 medical and health sciences, 0302 clinical medicine, Mastocytosis, Systemic, Risk Factors, Internal medicine, Humans, Medicine, Progression-free survival, Systemic mastocytosis, TRYPTASE, Aged, Retrospective Studies, National health, Serine-Arginine Splicing Factors, ANAPHYLAXIS, MUTATIONS, business.industry, Retrospective cohort study, Hematology, Middle Aged, Alkaline Phosphatase, Prognosis, medicine.disease, Stanford Cancer Institute, Progression-Free Survival, Repressor Proteins, International Prognostic Scoring System, 030220 oncology & carcinogenesis, Core Binding Factor Alpha 2 Subunit, Cohort, Female, business, KIT D816V, 030215 immunology
Abstract

[Background]: Several risk stratification models have been proposed in recent years for systemic mastocytosis but have not been directly compared. Here we designed and validated a risk stratification model for progression-free survival (PFS) and overall survival (OS) in systemic mastocytosis on the basis of all currently available prognostic factors, and compared its predictive capacity for patient outcome with that of other risk scores., [Methods]: We did a retrospective prognostic modelling study based on patients diagnosed with systemic mastocytosis between March 1, 1983, and Oct 11, 2019. In a discovery cohort of 422 patients from centres of the Spanish Network on Mastocytosis (REMA), we evaluated previously identified, independent prognostic features for prognostic effect on PFS and OS by multivariable analysis, and designed a global prognostic score for mastocytosis (GPSM) aimed at predicting PFS (GPSM-PFS) and OS (GPSM-OS) by including only those variables that showed independent prognostic value (p, [Findings]: Our GPSM-PFS and GPSM-OS models were based on unique combinations of independent prognostic factors for PFS (platelet count ≤100 × 109 cells per L, serum β2-microglobulin ≥2·5 μg/mL, and serum baseline tryptase ≥125 μg/L) and OS (haemoglobin ≤110 g/L, serum alkaline phosphatase ≥140 IU/L, and at least one mutation in SRSF2, ASXL1, RUNX1, or DNMT3A). The models showed clear discrimination between low-risk and high-risk patients in terms of worse PFS and OS prognoses in the discovery and validation cohorts, and further discrimination of intermediate-risk patients. The GPSM-PFS score was an accurate predictor of PFS in systemic mastocytosis (C-index 0·90 [95% CI 0·87–0·93], vs values ranging from 0·85 to 0·88 for pre-existing models), particularly in non-advanced systemic mastocytosis (C-index 0·85 [0·76–0·92], within the range for pre-existing models of 0·80 to 0·93). Additionally, the GPSM-OS score was able to accurately predict OS in the entire cohort (C-index 0·92 [0·89–0·94], vs 0·67 to 0·90 for pre-existing models), and showed some capacity to predict OS in advanced systemic mastocytosis (C-index 0·72 [0·66–0·78], vs 0·64 to 0·73 for pre-existing models)., [Interpretation]: All evaluated risk classifications predicted survival outcomes in systemic mastocytosis. The REMA-PFS and GPSM-PFS models for PFS, and the International Prognostic Scoring System for advanced systemic mastocytosis and GPSM-OS model for OS emerged as the most accurate models, indicating that robust prognostication might be prospectively achieved on the basis of biomarkers that are accessible in diagnostic laboratories worldwide., Carlos III Health Institute, European Regional Development Fund, Spanish Association of Mastocytosis and Related Diseases, Rare Diseases Strategy of the Spanish National Health System, Junta of Castile and León, Charles and Ann Johnson Foundation, Stanford Cancer Institute Innovation Fund, Austrian Science Fund.

Published
2021

29. Clinical impact and proposed application of molecular markers, genetic variants, and cytogenetic analysis in mast cell neoplasms: Status 2022

Author

Arock, Michel, primary, Hoermann, Gregor, additional, Sotlar, Karl, additional, Hermine, Olivier, additional, Sperr, Wolfgang R., additional, Hartmann, Karin, additional, Brockow, Knut, additional, Akin, Cem, additional, Triggiani, Massimo, additional, Broesby-Olsen, Sigurd, additional, Reiter, Andreas, additional, Gotlib, Jason, additional, Horny, Hans-Peter, additional, Orfao, Alberto, additional, Metcalfe, Dean D., additional, and Valent, Peter, additional

Published
2022
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30. High Frequency of Low-Count Monoclonal B-Cell Lymphocytosis in Hospitalized COVID-19 Patients

Author

Guillermo Oliva-Ariza, Blanca Fuentes-Herrero, Cristina Carbonell, Quentin Lecrevisse, Alba Pérez-Pons, Alba Torres-Valle, Julio Pozo, José Ángel Martín-Oterino, Óscar González-López, Amparo López-Bernús, Marta Bernal-Ribes, Moncef Belhassen-García, Oihane Pérez-Escurza, Martín Pérez-Andrés, Lourdes Vazquez, Guillermo Hernández-Pérez, Francisco Javier García Palomo, Pilar Leoz, Pilar Costa-Alba, Elena Pérez-Losada, Ana Yeguas, Miryam Santos Sánchez, Marta García-Blázquez, Francisco Javier Morán-Plata, Daniela Damasceno, Vitor Botafogo, Noemí Muñoz-García, Rafael Fluxa, Teresa Contreras-Sanfeliciano, Julia Almeida, Miguel Marcos, and Alberto Orfao

Subjects
Immunology, Cell Biology, Hematology, Biochemistry
Published
2022

31. High Frequency of Low-Count Monoclonal B-Cell Lymphocytosis in Hospitalized COVID-19 Patients

Author

Oliva-Ariza, Guillermo, primary, Fuentes-Herrero, Blanca, additional, Carbonell, Cristina, additional, Lecrevisse, Quentin, additional, Perez-Pons, Alba, additional, Torres-Valle, Alba, additional, Pozo, Julio, additional, Martín-Oterino, José-Ángel, additional, González López, Óscar, additional, López Bernús, Amparo, additional, Bernal Ribes, Marta, additional, Belhassen García, Moncef, additional, Perez-Escurza, Oihane, additional, Pérez Andrés, Martín, additional, Vazquez, Lourdes, additional, Hernández Pérez, Guillermo, additional, García Palomo, Francisco Javier, additional, Leoz, Pilar, additional, Costa Alba, Pilar, additional, Pérez Losada, María Elena, additional, Yeguas, Ana, additional, Santos Sánchez, Miryam, additional, García Blázquez, Marta, additional, Morán Plata, Francisco Javier, additional, Damasceno, Daniela, additional, Botafogo, Vitor, additional, Muñoz García, Noemí, additional, Fluxá, Rafael, additional, Contreras Sanfeliciano, Teresa, additional, Almeida, Julia, additional, Marcos, Miguel, additional, and Orfao, Alberto, additional

Published
2022
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32. Genomics Improves Risk Stratification of Adults with T-Cell Acute Lymphoblastic Leukemia Patients Enrolled in Measurable Residual Disease-Oriented Trials

Author

Gonzalez-Gil, Celia, primary, Morgades, Mireia, additional, Lopes, Thaysa, additional, Fuster, Francisco, additional, Garcia-Chica, Jesus, additional, Zhao, Ran, additional, Montesinos, Pau, additional, Torrent, Anna, additional, Diaz-Beya, Marina, additional, Coll, Rosa, additional, Hermosin, Lourdes, additional, Mercadal, Santiago, additional, Gonzalez-Campos, Jose, additional, Zamora, Lurdes, additional, Artola, Teresa, additional, Vall-llovera, Ferran, additional, Tormo, Mar, additional, Gil-Cortes, Cristina, additional, Barba, Pere, additional, Novo, Andres, additional, Ribera, Jordi, additional, Bernal, Teresa, additional, lopez, Paula, additional, Queipo, Mari-Paz, additional, Martinez-Sanchez, Pilar, additional, Gonzalez-Martinez, Teresa, additional, Cladera, Antonia, additional, Cervera, Jose, additional, Fernández-Martin, Rosa, additional, Ardaiz, Maria Angeles, additional, Vidal, Maria Jesus, additional, Baena, Angela, additional, Lopez-Bigas, Nuria, additional, Bigas, Anna, additional, Maciejewski, Jaroslaw, additional, Orfao, Alberto, additional, Ribera, Jose Maria, additional, and Genescà, Eulàlia, additional

Published
2022
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33. 3135 – CD34+CD19-CD22+ B-CELL PROGENITORS MIGHT UNDERLIE PHENOTYPIC ESCAPE IN PATIENTS TREATED WITH CD19-DIRECTED THERAPIES

Author

Menendez, Pablo, primary, Bueno, Clara, additional, Barrera, Susana, additional, Bataller, Alex, additional, Ortiz-Maldonado, Valentín, additional, Elliot, Natalina, additional, O'Byrne, Sorcha, additional, Wang, Gualing, additional, Rovira, Montse, additional, Gutierrez-Agüera, Francisco, additional, Trincado, Juan, additional, Gonzalez-Gonzalez, María, additional, Morgades, Mireia, additional, Sorigue, Marc, additional, Barcena, Paloma, additional, Zanetti, Samanta, additional, Torrebadell, Montse, additional, Vega, Nerea, additional, Rives, Susana, additional, Mallo, Mar, additional, Sole, Francesc, additional, Mead, Adam, additional, Roberts, Irene, additional, Thongjuea, Supat, additional, Psaila, Bethan, additional, Juan, Manel, additional, Delgado, Julio, additional, Urbano-Ispizúa, Alvaro, additional, Ribera, Jose, additional, Orfao, Alberto, additional, Roy, Anindita, additional, and Menendez, Pablo, additional

Published
2022
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34. ALL-268 Genetic Classification of B-Cell Precursor Adult Acute Lymphoblastic Leukemia Patients Enrolled in LAL19 Trial from the Pethema Group: Response to Treatment and Survival

Author

Jordi Ribera, Isabel Granada, Teresa González, Mireia Morgades, Ricardo Sánchez, Esperanza Such, Susana Barrena, Juana Ciudad, Beatriz Soriano, Rocío Benito, Gayane Avetisyan, Eva Lumbreras, Cristina Miguel, Sandra Santos, Lurdes Zamora, Mar Mallo, Eulàlia Genescà, Celia González, Thaysa Lopes, Jesús-María Hernández-Rivas, Alberto Orfao, and Josep Maria Ribera

Subjects
Adult, Cancer Research, Survival, Oncology, Genetic markers, B-cell, Hematology, Acute lymphoblastic leukemia, ALL
Abstract

Context: B-cell precursor acute lymphoblastic leukemia (BCP ALL) is a genetically heterogeneous neoplasm with >20 biologic subtypes. Each subtype shows specific genetic traits that determine relapse risk and patients' survival. Objectives: To establish the genetic subtype (primary alteration) of adult BCP ALL patients enrolled in the PETHEMA LAL19 trial (NCT 04179929) and to correlate them with measurable residual disease (MRD) level and survival. Patients and Methods: In the LAL19 trial (NCT04179929), Ph-negative patients (18–65 y) with MRD≥0.01% at day+35 or high-risk genetics receive alloHSCT and MRD

Published
2022

35. Poster: ALL-268 Genetic Classification of B-Cell Precursor Adult Acute Lymphoblastic Leukemia Patients Enrolled in LAL19 Trial from the Pethema Group: Response to Treatment and Survival

Author

Jordi Ribera, Isabel Granada, Teresa González, Mireia Morgades, Ricardo Sánchez, Esperanza Such, Susana Barrena, Juana Ciudad, Beatriz Soriano, Rocío Benito, Gayane Avetisyan, Eva Lumbreras, Cristina Miguel, Sandra Santos, Lurdes Zamora, Mar Mallo, Eulàlia Genescà, Celia González, Thaysa Lopes, Jesús-María Hernández-Rivas, Alberto Orfao, and Josep Maria Ribera

Subjects
Cancer Research, Oncology, Hematology
Published
2022

36. Selecting the Right Criteria and Proper Classification to Diagnose Mast Cell Activation Syndromes: A Critical Review

Author

Gülen, Theo, primary, Akin, Cem, additional, Bonadonna, Patrizia, additional, Siebenhaar, Frank, additional, Broesby-Olsen, Sigurd, additional, Brockow, Knut, additional, Niedoszytko, Marek, additional, Nedoszytko, Boguslaw, additional, Oude Elberink, Hanneke N.G., additional, Butterfield, Joseph H., additional, Sperr, Wolfgang R., additional, Alvarez-Twose, Ivan, additional, Horny, Hans-Peter, additional, Sotlar, Karl, additional, Schwaab, Juliana, additional, Jawhar, Mohamad, additional, Zanotti, Roberta, additional, Nilsson, Gunnar, additional, Lyons, Jonathan J., additional, Carter, Melody C., additional, George, Tracy I., additional, Hermine, Olivier, additional, Gotlib, Jason, additional, Orfao, Alberto, additional, Triggiani, Massimo, additional, Reiter, Andreas, additional, Hartmann, Karin, additional, Castells, Mariana, additional, Arock, Michel, additional, Schwartz, Lawrence B., additional, Metcalfe, Dean D., additional, and Valent, Peter, additional

Published
2021
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38. Improved Sézary cell detection and novel insights into immunophenotypic and molecular heterogeneity in Sézary syndrome

Author

Najidh, Safa, primary, Tensen, Cornelis P., additional, van der Sluijs-Gelling, Alita J., additional, Teodosio, Cristina, additional, Cats, Davy, additional, Mei, Hailiang, additional, Kuipers, Thomas B., additional, Out-Luijting, Jacoba J., additional, Zoutman, Willem H., additional, van Hall, Thorbald, additional, Orfao, Alberto, additional, Almeida, Julia, additional, van Dongen, Jacques J.M., additional, and Vermeer, Maarten H., additional

Published
2021
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39. PERFIL DE CÉLULAS B NA MEDULA ÓSSEA E SANGUE PERIFÉRICO DE PACIENTES COM MIELOMA MÚLTIPLO APÓS TRANSPLANTE AUTÓLOGO DE CÉLULAS TRONCO HEMATOPOIÉTICAS

Author

Barbosa, ES, primary, Pontes, RM, additional, Sanoja-Flores, L, additional, Flores-Montero, J, additional, Salgado, AB, additional, Puig, N, additional, Magalhães, RJP, additional, Mateos, MV, additional, Dongen, JJMV, additional, Maiolino, A, additional, Costa, ES, additional, and Orfao, A, additional

Published
2021
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40. THE ROLE OF LENALIDOMIDE MAINTENANCE AND MEASURABLE RESIDUAL DISEASE IN A REAL LIFE MULTIPLE MYELOMA TRANSPLANTED POPULATION RECEIVING DIFFERENT STRATEGIES GUIDED BY ACCESSIBLE TREATMENTS IN BRAZIL

Author

Salgado, ABDS, primary, Magalhães, RJP, additional, Pontes, RM, additional, Barbosa, E, additional, Pimenta, GS, additional, Flores-Montero, J, additional, Flores, LDCS, additional, Orfao, A, additional, Costa, ES, additional, and Maiolino, A, additional

Published
2021
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41. P-041: The role of Lenalidomide maintenance and measurable residual disease in a real-life multiple myeloma transplanted population receiving different strategies guided by accessible treatments in Brazil

Author

Salgado, Anna Beatriz, primary, Magalhães, Roberto J., additional, Pontes, Roberia, additional, Barbosa, Eduarda, additional, Pimenta, Glicinia, additional, Dutra, Helio, additional, Flores-Monteiro, Juan, additional, Flores, Luzalba, additional, Orfao, Alberto, additional, Costa, Elaine, additional, and Maiolino, Angelo, additional

Published
2021
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42. Adverse prognostic impact of complex karyotype (≥3 cytogenetic alterations) in adult T-cell acute lymphoblastic leukemia (T-ALL)

Author

Genescà, Eulàlia, primary, Morgades, Mireia, additional, González-Gil, Celia, additional, Fuster-Tormo, Francisco, additional, Haferlach, Claudia, additional, Meggendorfer, Manja, additional, Montesinos, Pau, additional, Barba, Pere, additional, Gil, Cristina, additional, Coll, Rosa, additional, Moreno, María-José, additional, Martínez-Carballeira, Daniel, additional, García-Cadenas, Irene, additional, Vives, Susana, additional, Ribera, Jordi, additional, González-Campos, José, additional, Díaz-Beya, Marina, additional, Mercadal, Santiago, additional, Artola, María-Teresa, additional, Cladera, Antonia, additional, Tormo, Mar, additional, Bermúdez, Arancha, additional, Vall-llovera, Ferran, additional, Martínez-Sánchez, Pilar, additional, Amigo, María-Luz, additional, Monsalvo, Silvia, additional, Novo, Andrés, additional, Cervera, Marta, additional, García-Guiñon, Antonio, additional, Ciudad, Juana, additional, Cervera, José, additional, Hernández-Rivas, Jesús-María, additional, Granada, Isabel, additional, Haferlach, Torsten, additional, Orfao, Alberto, additional, Solé, Francesc, additional, and Ribera, Josep-Maria, additional

Published
2021
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43. Automated identification of leukocyte subsets improves standardization of database-guided expert-supervised diagnostic orientation in acute leukemia: a EuroFlow study

Author

Lhermitte, Ludovic, primary, Barreau, Sylvain, additional, Morf, Daniela, additional, Fernandez, Paula, additional, Grigore, Georgiana, additional, Barrena, Susana, additional, de Bie, Maaike, additional, Flores-Montero, Juan, additional, Brüggemann, Monika, additional, Mejstrikova, Ester, additional, Nierkens, Stefan, additional, Burgos, Leire, additional, Caetano, Joana, additional, Gaipa, Giuseppe, additional, Buracchi, Chiara, additional, da Costa, Elaine Sobral, additional, Sedek, Lukasz, additional, Szczepański, Tomasz, additional, Aanei, Carmen-Mariana, additional, van der Sluijs-Gelling, Alita, additional, Delgado, Alejandro Hernández, additional, Fluxa, Rafael, additional, Lecrevisse, Quentin, additional, Pedreira, Carlos E., additional, van Dongen, Jacques J.M., additional, Orfao, Alberto, additional, van der Velden, Vincent H.J., additional, van Dongen, J. J.M., additional, Bitter, W.M., additional, Lubbers, B.R., additional, Teodosio, C.I., additional, Zlei, M., additional, van der Sluijs-Gelling, A.J., additional, de Bie, F., additional, de Bruin-Versteeg, S., additional, van der Burg, M., additional, Schilham, M.W., additional, van der Velden, V. H.J., additional, Langerak, A.W., additional, te Marvelde, J., additional, Bras, A.E., additional, Schilperoord-Vermeulen, J., additional, Jugooa, R., additional, Heezen, K.C., additional, Orfao, A., additional, Almeida, J., additional, Vidriales, M.B., additional, Flores-Montero, J., additional, Pérez-Andrés, M., additional, Matarraz, S., additional, Martín, L., additional, Lecrevisse, Q., additional, Pérez-Morán, J.J., additional, Puig, N., additional, Almeida, A. Medina, additional, Gomes da Silva, M., additional, Faria, T., additional, Brüggemann, M., additional, Ritgen, M., additional, Szczepanowski, M., additional, Kohlscheen, S., additional, Laqua, A., additional, Harbst, E., additional, Finke, J., additional, Asnafi, V., additional, Lhermitte, L., additional, Duroyon, E., additional, Trka, J., additional, Hrusak, O., additional, Kalina, T., additional, Mejstrikova, E., additional, Novakova, M., additional, Thurner, D., additional, Kanderova, V., additional, Szczepanski, T., additional, Sędek, L., additional, Bulsa, J., additional, Slota, L., additional, Kulis, J., additional, Pedreira, C.E., additional, da Costa, E. Sobral, additional, Nierkens, S., additional, de Jong, A., additional, de Koning, A., additional, Lima, M., additional, Santos, A.H., additional, Böttcher, S., additional, Lange, S., additional, Engelmann, R., additional, Paape, D., additional, Machka, C., additional, Gaipa, G., additional, Burracchi, C., additional, Bugarin, C., additional, Lopez-Granados, E., additional, del Pino Molina, L., additional, Campos-Guyotat, L., additional, Aanei, C., additional, Miguel, J. F. San, additional, Paiva, B., additional, Burgos, L., additional, Villamor-Casas, N., additional, Magnano, L., additional, Philippé, J., additional, Bonroy, C., additional, Denys, B., additional, Willems, A., additional, Breughe, P., additional, de Wolf, J., additional, Sousa, A.E., additional, Silva, S.L., additional, Fernandez, P., additional, and Morf, D., additional

Published
2021
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44. Personalized Management Strategies in Mast Cell Disorders: ECNM-AIM User’s Guide for Daily Clinical Practice

Author

Peter Valent, Karin Hartmann, Juliana Schwaab, Ivan Alvarez-Twose, Knut Brockow, Patrizia Bonadonna, Olivier Hermine, Marek Niedoszytko, Melody C. Carter, Gregor Hoermann, Wolfgang R. Sperr, Joseph H. Butterfield, Celalettin Ustun, Roberta Zanotti, Deepti H. Radia, Mariana Castells, Massimo Triggiani, Lawrence B. Schwartz, Alberto Orfao, Tracy I. George, Karl Sotlar, Jason Gotlib, Andreas Reiter, Hans-Peter Horny, Michel Arock, Cem Akin, Dean D. Metcalfe, Austrian Science Fund, Fundación de la Sociedad Española de Alergia e Inmunología Clínica, and National Institutes of Health (US)

Subjects
Allergy, MCAS, Tryptase, KIT, Immunoglobulin E, Personalized medicine, Proto-Oncogene Proteins c-kit, IgE, Mast cells, Mastocytosis, Systemic, Humans, Immunology and Allergy, Tryptases, Mast Cells, Mastocytosis
Abstract

Mastocytosis is a myeloid neoplasm defined by expansion and focal accumulation of clonal mast cells (MCs) in one or more organs. The disease exhibits a complex pathology and may be complicated by MC activation, bone abnormalities, neurological problems, gastrointestinal symptoms, and/or hematologic progression. The World Health Organization divides mastocytosis into cutaneous forms, systemic mastocytosis (SM) and MC sarcoma. In most patients with SM, somatic mutations in KIT are detected. Patients with indolent SM have a normal to near-normal life expectancy, whereas patients with advanced SM, including aggressive SM and MC leukemia, have a poor prognosis. In those with advanced SM, multiple somatic mutations and an associated hematologic neoplasm may be detected. Mediator-related symptoms can occur in any type of mastocytosis. Symptoms may be mild, severe, or even life-threatening. In patients with severe acute symptoms, an MC activation syndrome may be diagnosed. In these patients, relevant comorbidities include IgE-dependent and IgE-independent allergies. Management of patients with SM is an emerging challenge in daily practice and requires in-depth knowledge and a multidisciplinary and personalized approach with selection of appropriate procedures and interventions. In this article, we review the current knowledge on SM and MC activation syndrome, with emphasis on multidisciplinary aspects in diagnosis and patient-specific management. In addition, we provide a user’s guide for application of markers, algorithms, prognostic scores, and treatments for use in daily practice., This work was supported in part by the Austrian Science Fund (FWF; projects F4704 and P32470-B to P.V.) and the Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health (NIH) (to M.C.C. and D.D.M.). The content is solely the responsibility of the authors and does not represent the official views of the NIH.

Published
2022

45. ALL-154: t(1;19)(q23;p13) TCF3-PBX1 May Not Be an Intermediate-Risk Subtype in Adult B-Cell Precursor Acute Lymphoblastic Leukemia Patients Treated With MRD-Oriented Protocols from the PETHEMA Group

Author

Ribera, Jordi, primary, Morgades, Mireia, additional, Granada, Isabel, additional, Torrent, Anna, additional, Zamora, Lurdes, additional, González, Teresa, additional, Ciudad, Juana, additional, Barrena, Susana, additional, Such, Esperanza, additional, Avetisyan, Gayane, additional, Calasanz, Maria José, additional, Genescà, Eulàlia, additional, González-Gil, Celia, additional, Fuster-Tormo, Francisco, additional, Mercadal, Santiago, additional, Maluquer, Clara, additional, Coll, Rosa, additional, González-Campos, José, additional, Tormo, Mar, additional, García-Cadenas, Irene, additional, Nomdedeu, Josep, additional, Gil, Cristina, additional, Cervera, Marta, additional, Escoda, Lourdes, additional, Montesinos, Pau, additional, Barba, Pere, additional, Esteve, Jordi, additional, Díaz-Beyá, Marina, additional, Martínez-Sánchez, Pilar, additional, Martínez-López, Joaquín, additional, Novo, Andrés, additional, Queipo, M Paz, additional, Bermúdez, Arancha, additional, Bergua, Juan, additional, Olave, M Teresa, additional, De Rueda, Beatriz, additional, Artola, M Teresa, additional, Hernández-Rivas, Jesús M, additional, Orfao, Alberto, additional, and Ribera, Josep M, additional

Published
2021
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46. Poster: ALL-154: t(1;19)(q23;p13) TCF3-PBX1 May Not Be an Intermediate-Risk Subtype in Adult B-Cell Precursor Acute Lymphoblastic Leukemia Patients Treated With MRD-Oriented Protocols from the PETHEMA Group

Author

Ribera, Jordi, primary, Morgades, Mireia, additional, Granada, Isabel, additional, Torrent, Anna, additional, Zamora, Lurdes, additional, González, Teresa, additional, Ciudad, Juana, additional, Barrena, Susana, additional, Such, Esperanza, additional, Avetisyan, Gayane, additional, Calasanz, Maria José, additional, Genescà, Eulàlia, additional, González-Gil, Celia, additional, Fuster-Tormo, Francisco, additional, Mercadal, Santiago, additional, Maluquer, Clara, additional, Coll, Rosa, additional, González-Campos, José, additional, Tormo, Mar, additional, García-Cadenas, Irene, additional, Nomdedeu, Josep, additional, Gil, Cristina, additional, Cervera, Marta, additional, Escoda, Lourdes, additional, Montesinos, Pau, additional, Barba, Pere, additional, Esteve, Jordi, additional, Díaz-Beyá, Marina, additional, Martínez-Sánchez, Pilar, additional, Martínez-López, Joaquín, additional, Novo, Andrés, additional, Queipo, M Paz, additional, Bermúdez, Arancha, additional, Bergua, Juan, additional, Olave, M Teresa, additional, De Rueda, Beatriz, additional, Artola, M Teresa, additional, Hernández-Rivas, Jesús M, additional, Orfao, Alberto, additional, and Ribera, Josep M, additional

Published
2021
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47. A decision-tree approach for the differential diagnosis of chronic lymphoid leukemias and peripheral B-cell lymphomas

Author

Laura Oliveira Moraes, Carlos E. Pedreira, Susana Barrena, Antonio López, and Alberto Orfao

Subjects
Oncology, medicine.medical_specialty, Lymphoma, B-Cell, Computer science, Decision tree, Lymphoproliferative disorders, Health Informatics, Disease, Medical Oncology, Immunophenotyping, Flow cytometry, hemic and lymphatic diseases, Internal medicine, medicine, Humans, Medical diagnosis, B cell, Models, Statistical, medicine.diagnostic_test, Decision Trees, Reproducibility of Results, Flow Cytometry, medicine.disease, Peripheral blood, Leukemia, Lymphoid, Computer Science Applications, Lymphoma, Leukemia, Tree (data structure), medicine.anatomical_structure, Chronic Disease, Bone marrow, Differential diagnosis, Algorithms, Software
Abstract

Background and Objective Here we propose a decision-tree approach for the differential diagnosis of distinct WHO categories B-cell chronic lymphoproliferative disorders using flow cytometry data. Flow cytometry is the preferred method for the immunophenotypic characterization of leukemia and lymphoma, being able to process and register multiparametric data about tens of thousands of cells per second. Methods The proposed decision-tree is composed by logistic function nodes that branch throughout the tree into sets of (possible) distinct leukemia/lymphoma diagnoses. To avoid overfitting, regularization via the Lasso algorithm was used. The code can be run online at https://codeocean.com/2018/03/08/a-decision-tree-approach-for-the-differential-diagnosis-of-chronic-lymphoid-leukemias-and-peripheral-b-cell-lymphomas/ or downloaded from https://github.com/lauramoraes/bioinformatics-sourcecode to be executed in Matlab. Results The proposed approach was validated in diagnostic peripheral blood and bone marrow samples from 283 mature lymphoid leukemias/lymphomas patients. The proposed approach achieved 95% correctness in the cross-validation test phase (100% in-sample), 61% giving a single diagnosis and 34% (possible) multiple disease diagnoses. Similar results were obtained in an out-of-sample validation dataset. The generated tree reached the final diagnoses after up to seven decision nodes. Conclusions Here we propose a decision-tree approach for the differential diagnosis of mature lymphoid leukemias/lymphomas which proved to be accurate during out-of-sample validation. The full process is accomplished through seven binary transparent decision nodes.

Published
2019

48. P-041: The role of Lenalidomide maintenance and measurable residual disease in a real-life multiple myeloma transplanted population receiving different strategies guided by accessible treatments in Brazil

Author

Alberto Orfao, Roberia Pontes, Hélio S. Dutra, Elaine Sobral da Costa, Anna Beatriz Salgado, Roberto José Magalhaes, Luzalba Flores, Eduarda Barbosa, Glicinia Pimenta, Juan Flores-Monteiro, and Angelo Maiolino

Subjects
Oncology, Cancer Research, education.field_of_study, medicine.medical_specialty, Bortezomib, business.industry, Population, Significant difference, Protein profile, Hematology, Disease, medicine.disease, Internal medicine, Cohort, medicine, education, business, Multiple myeloma, Lenalidomide, medicine.drug
Abstract

Background Multiple myeloma (MM) treatment and monitoring with MRD-Next Generation Flow (NGF) has evolved fast in the last decade. Nevertheless, its incorporation by low-middle income countries remains challenging. Despite Lenalidomide maintenance (M-Len) after ASCT improves PFS and OS of MM, and MRD-NGF monitoring can discriminate patients (pts) with better outcomes, there is no data about these approaches in real-world pts in Brazil (BR) and Latin America. Methods Here we evaluated in two cohorts of pts guided by drug access, the benefit in outcomes of M-Len and MRD-NGF monitoring after ASCT. The study enrolled pts from public and private healthcare systems (HS). A total of 53 pts with symptomatic MM receiving up-front CTD n=27 or VCD n=26. All pts had a BM sample at D+100 for MRD-NGF following the EuroFlow SOPs with a limit of detection of 10-6 and a complete protein profile to meet the IMWG response and MRD criteria. Results Residual clonal plasma-cells were detected by MRD-NGF in 60% of all pts and in 44% of those in CR/sCR. MRD+ pts and showed a significant inferior outcome in this setting with median PFS of 26 months vs NR (p=0.05). Since Len was restricted to private HS, we evaluated its impact in a subset of 18 pts (30%), with a median treatment time of 20.5 months. In this group only, 2/18 (11%) cases progressed whereas in those with no M-Len, progression occurred in 19/35 (54%), with median PFS NR vs. 21 months (p=0.001). This benefit extended to OS, since in the M-len group had no deaths, in contrast to 11/35 (31%) (p=0.01) deaths without this drug. Combining the M-Len and MRD-NGF monitoring post ASCT allowed the recognition of distinct group outcomes: M-Len /MRD- (n=7) vs no M-Len/MRD+ (n=21) with median PFS NR vs 16 months (p=0.003). The benefit of maintenance improving disease control was clear among MRD+ pts (n=11) vs MRD+ pts with no M-Len (n=21): median PFS NR vs 16 months (p=0.002) and median OS NR in both groups but with a significant difference in the former (p=0.02). In our cohort, most pts admitted to the public HS had access to CTD without Len maintenance (n=24; 45%), while in the private received bortezomib in induction and M-Len post-transplant (n=15; 28%) with some pts having partial access with VCD/ no M-Len (n=11; 22%) or CTD/M-Len (n=3; 5%). Comparing strategies by drug access CTD/no-M-len in public vs VCD/M-len in private had an impact on both PFS (median of 16 months vs NR; p=0.003) and OS (median NR vs NR; p=0.02). Patients that had access to PI in induction without M-len also had worse outcomes: median PFS NR vs. 21 months for VCD/M-Len vs VCD/no M-Len, respectively (p=0.01), with a trend in OS (p=0.06). Conclusions In real-life, the use of M-Len post-ASCT is associated with better survival outcomes, MRD-NGF was a reproductible and powerful tool to discriminate pts at higher and earlier relapse risk. Inequity of drug access remains a hurdle in countries with constraints, particularly in public HS with a negative impact on survival of MM.

Published
2021

50. COVID-19 Vaccination in Mastocytosis: Recommendations of the European Competence Network on Mastocytosis (ECNM) and American Initiative in Mast Cell Diseases (AIM)

Author

Bonadonna, Patrizia, primary, Brockow, Knut, additional, Niedoszytko, Marek, additional, Elberink, Hanneke Oude, additional, Akin, Cem, additional, Nedoszytko, Boguslaw, additional, Butterfield, Joseph H., additional, Alvarez-Twose, Ivan, additional, Sotlar, Karl, additional, Schwaab, Juliana, additional, Jawhar, Mohamad, additional, Castells, Mariana, additional, Sperr, Wolfgang R., additional, Hermine, Olivier, additional, Gotlib, Jason, additional, Zanotti, Roberta, additional, Reiter, Andreas, additional, Broesby-Olsen, Sigurd, additional, Bindslev-Jensen, Carsten, additional, Schwartz, Lawrence B., additional, Horny, Hans-Peter, additional, Radia, Deepti, additional, Triggiani, Massimo, additional, Sabato, Vito, additional, Carter, Melody C., additional, Siebenhaar, Frank, additional, Orfao, Alberto, additional, Grattan, Clive, additional, Metcalfe, Dean D., additional, Arock, Michel, additional, Gulen, Theo, additional, Hartmann, Karin, additional, and Valent, Peter, additional

Published
2021
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