Evans syndrome in adults: an observational multicenter study
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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, Marıa Eva; McDonald, Vickie; Capecchi, Marco; Zaninoni, Anna; Consonni, Dario; Vos, Josephine Mathilde; Vianelli, Nicola; Chen, Frederick; Glenthøj, Andreas; Frederiksen, Henrik; Gonzalez-Lopez, Tomas Jose; Barcellini, Wilma.
I: Blood advances, Bind 5, Nr. 24, 2021, s. 5468-5478.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
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TY - JOUR
T1 - Evans syndrome in adults
T2 - an observational multicenter study
AU - Fattizzo, Bruno
AU - Michel, Marc
AU - Giannotta, Juri Alessandro
AU - Hansen, Dennis Lund
AU - Arguello, Maria
AU - Sutto, Emanuele
AU - Bianchetti, Nicola
AU - Patriarca, Andrea
AU - Cantoni, Silvia
AU - Mingot-Castellano, Marıa Eva
AU - McDonald, Vickie
AU - Capecchi, Marco
AU - Zaninoni, Anna
AU - Consonni, Dario
AU - Vos, Josephine Mathilde
AU - Vianelli, Nicola
AU - Chen, Frederick
AU - Glenthøj, Andreas
AU - Frederiksen, Henrik
AU - Gonzalez-Lopez, Tomas Jose
AU - Barcellini, Wilma
N1 - Publisher Copyright: © 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.
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
U2 - 10.1182/bloodadvances.2021005610
DO - 10.1182/bloodadvances.2021005610
M3 - Journal article
C2 - 34592758
AN - SCOPUS:85122411589
VL - 5
SP - 5468
EP - 5478
JO - Blood advances
JF - Blood advances
SN - 2473-9529
IS - 24
ER -
ID: 303581729