{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T10:45:26Z","timestamp":1788777926409,"version":"build-2803163510"},"reference-count":38,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,8,3]],"date-time":"2024-08-03T00:00:00Z","timestamp":1722643200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007747","name":"University of Utah","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007747","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2024,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Immune thrombocytopenia (ITP) and Evans syndrome (ES) are manifestations of immune dysregulation. Genetic variants in immune\u2010related genes have been identified in patients with ITP and especially ES. We aimed to explore familial autoimmunity in patients with ITP and ES to understand possible contributions to chronicity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>We assessed family history in two ways: via patient report for ITP and ES and by population\u2010based analysis using the Utah Population Database (UPDB) for ITP. A total of 266 patients with ITP and 21 patients with ES were identified via chart review, and 252 of the 266 patients with ITP were also identified in the UPDB.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Chart review showed familial autoimmunity in 29\/182 (15.9%) and 25\/84 (29.8%) of patients with newly diagnosed+persistent (nd+p) ITP and chronic ITP (cITP), respectively, (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.009). The UPDB analysis revealed that autoimmunity in relatives of patients with nd+pITP was higher than in relatives of controls (odds ratio [OR]: 1.69 [1.19\u20132.41], <jats:italic>p<\/jats:italic>\u00a0=\u00a0.004), but was not significantly increased in relatives of patients with cITP (OR 1.10 [0.63\u20131.92], <jats:italic>p<\/jats:italic>\u00a0=\u00a0.734). Incomplete family history in medical records likely contributed to the observed discrepancy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The findings suggest that familial autoimmunity may have a stronger association with the development of ITP rather than its duration. Twelve (57.1%) patients with ES reported autoimmunity in their relatives. UPDB analysis was omitted due to the small number of patients with ES. The use of population databases offers a unique opportunity to assess familial health and may provide clues about contributors to immune dysregulation features within families.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.31239","type":"journal-article","created":{"date-parts":[[2024,8,3]],"date-time":"2024-08-03T11:53:42Z","timestamp":1722686022000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Familial autoimmunity and risk of developing immune thrombocytopenia and Evans syndrome"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9204-702X","authenticated-orcid":false,"given":"Michell M.","family":"Lozano Chinga","sequence":"first","affiliation":[{"name":"Division of Allergy and Immunology Phoenix Children's Hospital Phoenix Arizona USA"},{"name":"Department of Child Health University of Arizona College of Medicine  Phoenix Arizona USA"},{"name":"Department of Pediatrics Creighton University  Phoenix Arizona USA"},{"name":"Department of Medicine Mayo Clinic College of Medicine and Science  Scottsdale Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2884-9247","authenticated-orcid":false,"given":"James B.","family":"Bussel","sequence":"additional","affiliation":[{"name":"Weill Cornell Medical School  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark N.","family":"Fluchel","sequence":"additional","affiliation":[{"name":"Seattle Children's Cancer and Blood Disorders Center Seattle Children's Hospital  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob","family":"Wilkes","sequence":"additional","affiliation":[{"name":"Intermountain Healthcare  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chong","family":"Zhang","sequence":"additional","affiliation":[{"name":"Division of Epidemiology Department of Internal Medicine University of Utah Health &amp; School of Medicine  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8712-4679","authenticated-orcid":false,"given":"Huong","family":"Meeks","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7222-4351","authenticated-orcid":false,"given":"Jessica A.","family":"Meznarich","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology Department of Pediatrics University of Utah  Salt Lake City Utah USA"},{"name":"Intermountain Healthcare Primary Children's Hospital  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,8,3]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2019000812"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2016.146373"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-04-570127"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.15125"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.1951.03810010058005"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm9123851"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-199709000-00005"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2022.869033"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2007.00914.x"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.13806"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms24055007"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2019.241968"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-11-887141"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2019000068"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-06-583260"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-022-01352-z"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2023009949"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2023011042"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.20989"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0006-341X.2000.00645.x"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-022-05486-5"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00467-022-05482-9"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1182\/hematology.2020000173"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006480"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26900"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2020.271106"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPH.0000000000001836"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020004888"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102019\u2010130418"},{"key":"e_1_2_9_31_1","unstructured":"Bureau USC.Hispanic or Latino origin by specific origin: 2021 American Community Survey estimates.United States Census Bureau. 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