{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T14:55:20Z","timestamp":1791816920342,"version":"4.3.4"},"reference-count":24,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2018,2,8]]},"abstract":"<jats:p>Factor XI (FXI) deficiency is an autosomal disorder which manifests as bleeding of varying severity. While homozygotes typically experience more dramatic bleeding symptoms, heterozygotes may experience clinically significant bleeding following surgical procedures or trauma, and therefore the condition is not purely recessive. The clinical significance of FXI deficiency is complicated in that FXI levels do not correlate well with bleeding severity, and in fact the bleeding risk is variable even for an individual in response to different haemostatic challenges. We present the case of a 74-year-old man of Ashkenazi Jewish heritage with a family and personal history of bleeding during surgical procedures, who presented with excessive bleeding following total thyroidectomy. He was found to have a FXI level of 52% (low normal). Genetic testing revealed that he was heterozygous for the c.403G&gt;T mutation. This case demonstrates successful work-up and perioperative management of a patient with FXI deficiency.<\/jats:p>","DOI":"10.1136\/bcr-2017-222434","type":"journal-article","created":{"date-parts":[[2018,2,21]],"date-time":"2018-02-21T08:10:37Z","timestamp":1519200637000},"page":"bcr-2017-222434","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Successful perioperative management in a patient with factor XI deficiency"],"prefix":"10.1136","volume":"2018","author":[{"given":"Margaret L","family":"McCarthy","sequence":"first","affiliation":[{"name":"Georgetown University School of Medicine, Washington DC, Columbia, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah M","family":"Ordway","sequence":"additional","affiliation":[{"name":"Internal Medicine, Walter Reed National Military Medical Center, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryan M","family":"Jones","sequence":"additional","affiliation":[{"name":"Hematology-Oncology, Walter Reed National Military Medical Center, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy G","family":"Perkins","sequence":"additional","affiliation":[{"name":"Hematology-Oncology, Walter Reed National Military Medical Center, Bethesda, Maryland, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,2,21]]},"reference":[{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.1","doi-asserted-by":"publisher","DOI":"10.3181\/00379727-82-20057"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.2","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1182\/blood.V10.2.120.120","article-title":"Plasma thromboplastin antecedent (PTA) deficiency; clinical, coagulation, therapeutic and hereditary aspects of a new hemophilia-like disease","volume":"10","author":"Rosenthal","year":"1955","journal-title":"Blood"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08945.x"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.4","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03395.x"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.5","doi-asserted-by":"crossref","unstructured":"Gailani D , Wheeler AP , Neff AT . Rare coagulation factor deficiencies. Hoffman R , ed. Hematology: basic principles and practice. 7th\u00a0edn. Philadelphia, PA: Elsevier Inc, 2018:2034\u201350.","DOI":"10.1016\/B978-0-323-35762-3.00137-2"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.6","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/bjh.14227","article-title":"The safety of chronic antithrombotic therapy in patients with factor XI deficiency","volume":"179","author":"Chapin","year":"2017","journal-title":"Br J Haematol"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.7","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2004.00992.x"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.8","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1160\/TH07-04-0246","article-title":"Factor XI in haemostasis and thrombosis: past, present and future","volume":"98","author":"Seligsohn","year":"2007","journal-title":"Thromb Haemost"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.9","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12682"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.10","unstructured":"Factor XI. Factor XI Deficiency (Hemophilia C, Plasma Thromboplastin Antecedent (PTA) Deficiency, Rosenthal Syndrome). https:\/\/www.hemophilia.org\/Bleeding-Disorders\/Types-of-Bleeding-Disorders\/Other-Factor-Deficiencies\/Factor-XI (accessed 4 Feb 2017)."},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.11","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1055\/s-0037-1615132","article-title":"Factor XI and protection of the fibrin clot against lysis:\u00a0a role for the intrinsic pathway of coagulation in fibrinolysis","volume":"80","author":"Bouma","year":"1998","journal-title":"Thromb Haemost"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.12","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1353391"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.13","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1111\/j.1365-2516.2008.01667.x","article-title":"Factor XI deficiency","volume":"14","author":"Gomez","year":"2008","journal-title":"Haemophilia"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.14","doi-asserted-by":"crossref","DOI":"10.1182\/blood.V128.22.3796.3796","article-title":"Perioperative management for congenital factor XI deficiency; a systematic review [Abstract]","volume":"128","author":"Chai-Adisaksopha","year":"2016","journal-title":"Blood"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.15","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1160\/TH09-03-0172","article-title":"Recombinant activated factor VII and tranexamic acid are haemostatically effective during major surgery in factor XI-deficient patients with inhibitor antibodies","volume":"102","author":"Livnat","year":"2009","journal-title":"Thromb Haemost"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.16","doi-asserted-by":"publisher","DOI":"10.1055\/s-0033-1353420"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.17","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.20.7667"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.18","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1111\/j.1538-7836.2009.03395.x","article-title":"Factor XI deficiency in humans","volume":"7 Suppl 1","author":"Seligsohn","year":"2009","journal-title":"J Thromb Haemost"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.19","doi-asserted-by":"publisher","DOI":"10.1172\/JCI119412"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.20","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1055\/s-0037-1614902","article-title":"Factor XI dependent and independent activation of thrombin activatable fibrinolysis inhibitor (TAFI) in plasma associated with clot formation","volume":"82","author":"Bouma","year":"1999","journal-title":"Thromb Haemost"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.21","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199107183250303"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.22","doi-asserted-by":"publisher","DOI":"10.1373\/clinchem.2015.248625"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.23","doi-asserted-by":"crossref","first-page":"13:1","DOI":"10.1186\/1477-9560-13-1","article-title":"A general review of major global coagulation assays: thrombelastography, thrombin generation test and clot waveform analysis","volume":"13","author":"Lanc\u00e9","year":"2015","journal-title":"Thromb J"},{"key":"2025082623445466000_2018.feb21_1.bcr-2017-222434.24","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1055\/s-0028-1104546","article-title":"Standardization and clinical utility of thrombin: generation assays","volume":"34","author":"Berntorp","year":"2008","journal-title":"Semin Thromb Hemost"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2017-222434","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T06:45:07Z","timestamp":1756277107000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2017-222434"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2,21]]},"references-count":24,"alternative-id":["10.1136\/bcr-2017-222434"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2017-222434","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,2,21]]}}}