Evans syndrome (ES) is rare and mostly treated on a "case-by-case" basis and no guidelines are available. With the aim of assessing disease awareness and current management of adult ES, a structured survey was administered to 64 clinicians from 50 Italian participating centers. Clinicians had to be involved in the management of autoimmune cytopenias and were enrolled into the ITP-NET initiative. The survey included domains on epidemiology, diagnosis, and therapy of ES and was designed to capture current practice and suggested work-up and management. Thirty clinicians who had followed a median of 5 patients (1-45)/15 years responded. The combination of AIHA plus ITP was more common than the ITP/AIHA with neutropenia (p < .001) and 25% of patients had an associated condition, including lymphoproliferative syndromes, autoimmune diseases, or primary immunodeficiencies. The agreement of clinicians for each diagnostic test is depicted (i.e., 100% for blood count and DAT; only 40% for anti-platelets and anti-neutrophils; 77% for bone marrow evaluation). Most clinicians reported that ES requires a specific approach compared to isolated autoimmune cytopenias, due to either a more complex pathogenesis and a higher risk of relapse and thrombotic and infectious complications. The heterogeneity of treatment choices among different physicians suggests the need for broader harmonization.
Evans syndrome: Disease awareness and clinical management in a nation-wide ITP-NET survey / Fattizzo, Bruno; Carrai, Valentina; Crugnola, Monica; Baldacci, Erminia; Bellini, Marta; Bosi, Costanza; Buzzatti, Elisa; Caramazza, Domenica; Carli, Giuseppe; Carpenedo, Monica; Clissa, Cristina; Danesin, Cristina; De Paolis, Maria Rosaria; Giannotta, Juri Alessandro; Innao, Vanessa; Marchetti, Monia; Markovic, Uros; Morotti, Alessandro; Napolitano, Mariasanta; Patriarca, Andrea; Pettine, Loredana; Poloni, Antonella; Rivolti, Elena; Rossi, Elena; Santeremo, Teresa Maria; Santoro, Cristina; Zannier, Maria Elena; Zaja, Francesco; Cantoni, Silvia; Palandri, Francesca; De Stefano, Valerio. - In: EUROPEAN JOURNAL OF HAEMATOLOGY. - ISSN 0902-4441. - (2024). [Epub ahead of print] [10.1111/ejh.14256]
Evans syndrome: Disease awareness and clinical management in a nation-wide ITP-NET survey
Poloni, Antonella;
2024-01-01
Abstract
Evans syndrome (ES) is rare and mostly treated on a "case-by-case" basis and no guidelines are available. With the aim of assessing disease awareness and current management of adult ES, a structured survey was administered to 64 clinicians from 50 Italian participating centers. Clinicians had to be involved in the management of autoimmune cytopenias and were enrolled into the ITP-NET initiative. The survey included domains on epidemiology, diagnosis, and therapy of ES and was designed to capture current practice and suggested work-up and management. Thirty clinicians who had followed a median of 5 patients (1-45)/15 years responded. The combination of AIHA plus ITP was more common than the ITP/AIHA with neutropenia (p < .001) and 25% of patients had an associated condition, including lymphoproliferative syndromes, autoimmune diseases, or primary immunodeficiencies. The agreement of clinicians for each diagnostic test is depicted (i.e., 100% for blood count and DAT; only 40% for anti-platelets and anti-neutrophils; 77% for bone marrow evaluation). Most clinicians reported that ES requires a specific approach compared to isolated autoimmune cytopenias, due to either a more complex pathogenesis and a higher risk of relapse and thrombotic and infectious complications. The heterogeneity of treatment choices among different physicians suggests the need for broader harmonization.File | Dimensione | Formato | |
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