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For decades, the evolution of Evans syndrome (ES) has carried a poor prognosis and often resulted in chronic steroid exposure, multiple immune suppressing medications directed against T or B lymphocytes, and splenectomy. This paper presents a new view of ES based on recent advances in genomics which begin to classify patients based on their underlying molecular variants in previously described primary immune disorders. This has opened up new avenues of targeted therapy or bone marrow transplant at rather than broad long\u2010term immune suppression or splenectomy. Importantly, recent studies of the full lifespan of ES suggest that at least 80% of those paediatric patients will progress to various clinical or biological immunopathological manifestations with age despite the resolution of their cytopenias. Those patients merit long\u2010term follow\u2010up and monitoring in dedicated transition programs to improve outcome at the adult age.<\/jats:p>","DOI":"10.1111\/bjh.19073","type":"journal-article","created":{"date-parts":[[2023,9,22]],"date-time":"2023-09-22T03:20:29Z","timestamp":1695352829000},"page":"28-35","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Paediatric\u2010onset Evans syndrome: Breaking away from refractory immune thrombocytopenia"],"prefix":"10.1111","volume":"203","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0231-4460","authenticated-orcid":false,"given":"Nathalie","family":"Aladjidi","sequence":"first","affiliation":[{"name":"Centre de R\u00e9f\u00e9rence National des Cytop\u00e9nies Auto\u2010immunes de l'Enfant (CEREVANCE)  Bordeaux France"},{"name":"Pediatric Hemato\u2010Immunology, CIC1401, INSERM CICP Bordeaux University Hospital  Bordeaux 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