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Evans syndrome in adults: an observational multicenter study

Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferat...

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Autores principales: Fattizzo, Bruno, Michel, Marc, Giannotta, Juri Alessandro, Hansen, Dennis Lund, Arguello, Maria, Sutto, Emanuele, Bianchetti, Nicola, Patriarca, Andrea, Cantoni, Silvia, Mingot-Castellano, María Eva, McDonald, Vickie, Capecchi, Marco, Zaninoni, Anna, Consonni, Dario, Vos, Josephine Mathilde, Vianelli, Nicola, Chen, Frederick, Glenthøj, Andreas, Frederiksen, Henrik, González-López, Tomás José, Barcellini, Wilma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society of Hematology 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8714709/
https://www.ncbi.nlm.nih.gov/pubmed/34592758
http://dx.doi.org/10.1182/bloodadvances.2021005610
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author Fattizzo, Bruno
Michel, Marc
Giannotta, Juri Alessandro
Hansen, Dennis Lund
Arguello, Maria
Sutto, Emanuele
Bianchetti, Nicola
Patriarca, Andrea
Cantoni, Silvia
Mingot-Castellano, María Eva
McDonald, Vickie
Capecchi, Marco
Zaninoni, Anna
Consonni, Dario
Vos, Josephine Mathilde
Vianelli, Nicola
Chen, Frederick
Glenthøj, Andreas
Frederiksen, Henrik
González-López, Tomás José
Barcellini, Wilma
author_facet Fattizzo, Bruno
Michel, Marc
Giannotta, Juri Alessandro
Hansen, Dennis Lund
Arguello, Maria
Sutto, Emanuele
Bianchetti, Nicola
Patriarca, Andrea
Cantoni, Silvia
Mingot-Castellano, María Eva
McDonald, Vickie
Capecchi, Marco
Zaninoni, Anna
Consonni, Dario
Vos, Josephine Mathilde
Vianelli, Nicola
Chen, Frederick
Glenthøj, Andreas
Frederiksen, Henrik
González-López, Tomás José
Barcellini, Wilma
author_sort Fattizzo, Bruno
collection PubMed
description Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations, and outcomes. In this retrospective international study, we analyzed 116 adult patients followed at 13 European tertiary centers, focusing on treatment requirements, occurrence of complications, and death. ES was secondary to or associated with underlying conditions in 24 cases (21%), mainly other autoimmune diseases and hematologic neoplasms. Bleeding occurred in 42% of patients, mainly low grade and at ITP onset. Almost all patients received first-line treatment (steroids with or without intravenous immunoglobulin), and 23% needed early additional therapy for primary refractoriness. Additional therapy lines included rituximab, splenectomy, immunosuppressants, thrombopoietin receptor agonists, and others, with response rates >80%. However, a remarkable number of relapses occurred, requiring ≥3 therapy lines in 54% of cases. Infections and thrombotic complications occurred in 33% and 21% of patients, respectively, mainly grade ≥3, and correlated with the number of therapy lines. In addition to age, other factors negatively affecting survival were severe anemia at onset and occurrence of relapse, infection, and thrombosis. These data show that adult ES is often severe and marked by a relapsing clinical course and potentially fatal complications, pinpointing the need for high clinical awareness, prompt therapy, and anti-infectious/anti-thrombotic prophylaxis.
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spelling pubmed-87147092021-12-29 Evans syndrome in adults: an observational multicenter study Fattizzo, Bruno Michel, Marc Giannotta, Juri Alessandro Hansen, Dennis Lund Arguello, Maria Sutto, Emanuele Bianchetti, Nicola Patriarca, Andrea Cantoni, Silvia Mingot-Castellano, María Eva McDonald, Vickie Capecchi, Marco Zaninoni, Anna Consonni, Dario Vos, Josephine Mathilde Vianelli, Nicola Chen, Frederick Glenthøj, Andreas Frederiksen, Henrik González-López, Tomás José Barcellini, Wilma Blood Adv Platelets and Thrombopoiesis Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations, and outcomes. In this retrospective international study, we analyzed 116 adult patients followed at 13 European tertiary centers, focusing on treatment requirements, occurrence of complications, and death. ES was secondary to or associated with underlying conditions in 24 cases (21%), mainly other autoimmune diseases and hematologic neoplasms. Bleeding occurred in 42% of patients, mainly low grade and at ITP onset. Almost all patients received first-line treatment (steroids with or without intravenous immunoglobulin), and 23% needed early additional therapy for primary refractoriness. Additional therapy lines included rituximab, splenectomy, immunosuppressants, thrombopoietin receptor agonists, and others, with response rates >80%. However, a remarkable number of relapses occurred, requiring ≥3 therapy lines in 54% of cases. Infections and thrombotic complications occurred in 33% and 21% of patients, respectively, mainly grade ≥3, and correlated with the number of therapy lines. In addition to age, other factors negatively affecting survival were severe anemia at onset and occurrence of relapse, infection, and thrombosis. These data show that adult ES is often severe and marked by a relapsing clinical course and potentially fatal complications, pinpointing the need for high clinical awareness, prompt therapy, and anti-infectious/anti-thrombotic prophylaxis. American Society of Hematology 2021-12-16 /pmc/articles/PMC8714709/ /pubmed/34592758 http://dx.doi.org/10.1182/bloodadvances.2021005610 Text en © 2021 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.
spellingShingle Platelets and Thrombopoiesis
Fattizzo, Bruno
Michel, Marc
Giannotta, Juri Alessandro
Hansen, Dennis Lund
Arguello, Maria
Sutto, Emanuele
Bianchetti, Nicola
Patriarca, Andrea
Cantoni, Silvia
Mingot-Castellano, María Eva
McDonald, Vickie
Capecchi, Marco
Zaninoni, Anna
Consonni, Dario
Vos, Josephine Mathilde
Vianelli, Nicola
Chen, Frederick
Glenthøj, Andreas
Frederiksen, Henrik
González-López, Tomás José
Barcellini, Wilma
Evans syndrome in adults: an observational multicenter study
title Evans syndrome in adults: an observational multicenter study
title_full Evans syndrome in adults: an observational multicenter study
title_fullStr Evans syndrome in adults: an observational multicenter study
title_full_unstemmed Evans syndrome in adults: an observational multicenter study
title_short Evans syndrome in adults: an observational multicenter study
title_sort evans syndrome in adults: an observational multicenter study
topic Platelets and Thrombopoiesis
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8714709/
https://www.ncbi.nlm.nih.gov/pubmed/34592758
http://dx.doi.org/10.1182/bloodadvances.2021005610
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