{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T16:48:00Z","timestamp":1772556480882,"version":"3.50.1"},"reference-count":22,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T00:00:00Z","timestamp":1772496000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Reports"],"abstract":"<jats:p>Background: Pediatric-onset Evans syndrome (pES) is a rare autoimmune disorder defined by the coexistence or sequential development of immune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA), frequently accompanied by autoimmune neutropenia (AIN) and characterized by a relapsing, multilineage course. Increasing evidence suggests that pES may represent a broader immune dysregulation phenotype rather than an isolated hematologic disorder. Methods: We conducted a retrospective, single-center study of children diagnosed with pES and followed for up to 13 years at a tertiary referral center. Clinical data regarding hematologic evolution, extra-hematological immunopathological manifestations, treatment requirements, infectious complications, and genetic findings were analyzed descriptively. Results: Six children (4 males) were included, with a median age at first cytopenia of 7 years (range 3\u201315) and a median follow-up of 8 years (range 1\u201313). ITP preceded AIHA in 3\/6 patients (50%), one patient (16.7%) developed AIHA first, and two (33.3%) showed partial or evolving multilineage disease with DAT positivity prior to overt hemolysis. AIN occurred in 3\/6 patients (50%). Extra-hematological immunopathological manifestations occurred in 5\/6 patients (83.3%), with two (33.3%) developing more than one. Second-line therapy was required in 3\/6 patients (50%). Infectious episodes occurred in 83.3% of patients, predominantly viral or mild bacterial infections, with no life-threatening events. Whole-exome sequencing performed in three patients identified a heterozygous TNFAIP3 variant of uncertain significance in one case; no pathogenic variants were detected. Conclusions: pES demonstrates clinical heterogeneity, frequent multilineage cytopenia, and substantial extra-hematological immune involvement. Multisystem manifestations may be associated with increased treatment burden. Long-term multidisciplinary monitoring and cautious interpretation of genetic findings are essential for individualized pediatric care.<\/jats:p>","DOI":"10.3390\/pediatric18020034","type":"journal-article","created":{"date-parts":[[2026,3,3]],"date-time":"2026-03-03T12:48:56Z","timestamp":1772542136000},"page":"34","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pediatric Evans Syndrome as a Multisystem Immune Disorder: A 13-Year Longitudinal Experience from a Single Academic Center"],"prefix":"10.3390","volume":"18","author":[{"given":"Dimitrios","family":"Karamitsos","sequence":"first","affiliation":[{"name":"Faculty of Medicine, University of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ioanna Paraskevi","family":"Papandrea","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nikoletta","family":"Rokidi","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ioanna","family":"Saougou","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University Hospital of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1244-375X","authenticated-orcid":false,"given":"Chrysoula","family":"Kosmeri","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University Hospital of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0142-6110","authenticated-orcid":false,"given":"Alexandros","family":"Makis","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University Hospital of Ioannina, 45500 Ioannina, Greece"},{"name":"Child Health Department, Faculty of Medicine, University of Ioannina, 45500 Ioannina, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,3,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1001\/archinte.1951.03810010058005","article-title":"Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology","volume":"87","author":"Evans","year":"1951","journal-title":"A.M.A. Arch. 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