{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T19:03:49Z","timestamp":1787166229944,"version":"build-2736575974"},"reference-count":49,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T00:00:00Z","timestamp":1695945600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>Acquired factor XIII (FXIII) deficiency is a very rare haemostatic defect that can be either immune (rare development of an autoantibody targeting FXIII epitopes) or nonimmune (diminished synthesis or increased consumption of the same factor). The aim of this study is to review the symptomatology, the diagnostic method used, but above all to determine the most frequently used and potentially most effective treatment for acquired FXIII deficiency.<\/jats:p>\n                    <jats:p>PubMed, Medline, embase\/Ovid databases were queried from 1 January 2012 to 3 April 2022. Data extraction was performed using the keywords \u2018Acquired FXIII deficiency.\u2019<\/jats:p>\n                    <jats:p>The systematic search identified 474 records. After screening titles and abstracts, only 36 articles met the eligibility criteria.<\/jats:p>\n                    <jats:p>The mean age of all patients was 57.6 [range, 1\u201384] years. The male to female ratio was 35\u200a:\u200a25.<\/jats:p>\n                    <jats:p>The majority of cases described were due an autoimmune reaction with antibody production (24 manuscripts), only six manuscripts described consumption. The most prevalent symptoms were local haematoma (31).<\/jats:p>\n                    <jats:p>Six cases died, two from haemorrhagic shock, two from haemorrhagic stroke, one from respiratory distress, and 1 from septic shock.<\/jats:p>\n                    <jats:p>Given the patient outcomes, this review confirms that the most appropriate treatment consists of one of the following elements or a combination of several of these elements: FXIII concentrate, corticosteroids, cyclophosphamide, rituximab for autoimmune cases, and FXIII concentrate supplementation only in case of consumption.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1097\/ea9.0000000000000035","type":"journal-article","created":{"date-parts":[[2023,9,29]],"date-time":"2023-09-29T03:01:34Z","timestamp":1695956494000},"page":"e0035","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":9,"title":["Acquired factor XIII deficiency"],"prefix":"10.1097","volume":"2","author":[{"given":"Olivier","family":"Duranteau","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guney","family":"Tatar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Demulder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Turgay","family":"Tuna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2023,10]]},"reference":[{"key":"R1-20240826","doi-asserted-by":"crossref","first-page":"494","DOI":"10.2741\/4731","article-title":"The regulatory role of coagulation factors in vascular function","volume":"24","author":"Lagrange","year":"2019","journal-title":"Front Biosci (Landmark Ed)"},{"key":"R2-20240826","doi-asserted-by":"crossref","first-page":"1699","DOI":"10.3390\/jcm11061699","article-title":"Measuring factor XIII inhibitors in patients with factor XIII deficiency: a case report and systematic review of current practices in Japan","volume":"11","author":"Amano","year":"2022","journal-title":"J Clin Med"},{"key":"R3-20240826","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1186\/s13054-022-03940-2","article-title":"The impact of acquired coagulation factor XIII deficiency in traumatic bleeding and wound healing","volume":"26","author":"Kleber","year":"2022","journal-title":"Crit Care"},{"key":"R4-20240826","first-page":"48","article-title":"Acquired FXIII deficiency is associated with high morbidity","volume":"122","author":"Duque","year":"2021","journal-title":"Thromb Haemost"},{"key":"R5-20240826","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1111\/j.1365-2516.2012.02763.x","article-title":"A case of acquired FXIII deficiency with severe bleeding symptoms","volume":"18","author":"Hayashi","year":"2012","journal-title":"Haemophilia"},{"key":"R6-20240826","doi-asserted-by":"crossref","first-page":"724","DOI":"10.1016\/j.transci.2018.10.013","article-title":"Acquired factor XIII deficiency: a review","volume":"57","author":"Yan","year":"2018","journal-title":"Transfus Apher Sci"},{"key":"R7-20240826","doi-asserted-by":"crossref","first-page":"38","DOI":"10.7453\/gahmj.2013.008","article-title":"The CARE guidelines: consensus-based clinical case reporting guideline development","volume":"2","author":"Gagnier","year":"2013","journal-title":"Glob Adv Health Med"},{"key":"R8-20240826","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.tmrv.2016.04.001","article-title":"Acquired factor XIII inhibitor in hospitalized and perioperative patients: a systematic review of case reports and case series","volume":"30","author":"Tone","year":"2016","journal-title":"Transfus Med Rev"},{"key":"R9-20240826","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/1471-2288-4-22","article-title":"A systematic review of the content of critical appraisal tools","volume":"4","author":"Katrak","year":"2004","journal-title":"BMC Med Res Methodol"},{"key":"R10-20240826","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1111\/hae.13205","article-title":"Severe bleeding diatheses in an elderly patient with combined type autoantibody against factor XIII A subunit; 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