RT Journal Article
T1 Evans syndrome in adults: an observational multicenter study
A1 Fattizzo, Bruno
A1 Michel, Marc
A1 Alessandro Giannotta, Juri
A1 Lund Hansen, Dennis
A1 Arguello, Maria
A1 Sutto, Emanuele
A1 Bianchetti, Nicola
A1 Patriarca, Andrea
A1 Cantoni, Silvia
A1 Eva Mingot-Castellano, Maria
A1 McDonald, Vickie
A1 Capecchi, Marco
A1 Zaninoni, Anna
A1 Consonni, Dario
A1 Mathilde Vos, Josephine
A1 Vianelli, Nicola
A1 Chen, Frederick
A1 Glenthoj, Andreas
A1 Frederiksen, Henrik
A1 Jose Gonzalez-Lopez, Tomas
A1 Barcellini, Wilma
K1 Thrombocytopenic purpura
K1 Hemolytic-anemia
K1 Itp
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.
PB Elsevier
SN 2473-9529
YR 2021
FD 2021-12-17
LK https://hdl.handle.net/10668/27462
UL https://hdl.handle.net/10668/27462
LA en
DS RISalud
RD Apr 10, 2025