{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,8]],"date-time":"2026-11-08T04:13:34Z","timestamp":1794111214544,"version":"4.8.0"},"reference-count":91,"publisher":"American Society of Hematology","issue":"15","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,4,11]]},"abstract":"<jats:sec>\n                    <jats:title>Abstract<\/jats:title>\n                    <jats:p>Factor XI (FXI) deficiency is a rare bleeding disorder that presents complex challenges in patient assessment and bleeding risk management. Despite generally causing mild to moderate bleeding symptoms, clinical manifestations can vary, and bleeding tendency does not always correlate with FXI plasma levels or genotype. Our manuscript delves into the age-related nuances of FXI deficiency across an individual's lifespan. We emphasize issues faced by specific groups, including neonates and females of reproductive age experiencing abnormal uterine bleeding and postpartum hemorrhage. Older patients present unique challenges and concerns related to the management of bleeding as well as thrombotic complications. The current assortment of diagnostic laboratory assays shows limited success in predicting bleeding risk in the perisurgical setting of patients with FXI deficiency. This review explores the intricate interplay between individual bleeding profiles, surgical sites, and FXI activity levels. We also evaluate the accuracy of existing laboratory assays in predicting bleeding and discuss the potential role of investigational global assays in perioperative assessment. Furthermore, we outline our suggested diagnostic approach to refine treatment strategies and decision making. Available treatment options are presented, including antifibrinolytics, replacement products, and recombinant activated FVII. Finally, we discuss promising nonreplacement therapies for the treatment of rare bleeding disorders that can potentially address the challenges faced when managing FXI deficiency\u2013related bleeding complications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1182\/blood.2023020721","type":"journal-article","created":{"date-parts":[[2024,1,9]],"date-time":"2024-01-09T17:07:33Z","timestamp":1704820053000},"page":"1455-1464","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":46,"title":["Factor XI deficiency: phenotypic age-related considerations and clinical approach towards bleeding risk assessment"],"prefix":"10.1182","volume":"143","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8329-8409","authenticated-orcid":false,"given":"Assaf Arie","family":"Barg","sequence":"first","affiliation":[{"name":"National Hemophilia Center, Coagulation Unit and Amalia Biron Research Institution of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Israel; and Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1279-2557","authenticated-orcid":false,"given":"Tami","family":"Livnat","sequence":"additional","affiliation":[{"name":"National Hemophilia Center, Coagulation Unit and Amalia Biron Research Institution of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Israel; and Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3494-9823","authenticated-orcid":false,"given":"Gili","family":"Kenet","sequence":"additional","affiliation":[{"name":"National Hemophilia Center, Coagulation Unit and Amalia Biron Research Institution of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer, Israel; and Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"issue":"4","key":"2024041115212086200_bib1","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1182\/blood-2017-04-780148","article-title":"Exploring the global landscape of genetic variation in coagulation factor XI deficiency","volume":"130","author":"Asselta","year":"2017","journal-title":"Blood"},{"issue":"6","key":"2024041115212086200_bib2","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1055\/s-0033-1353420","article-title":"Congenital factor XI deficiency: an update","volume":"39","author":"Duga","year":"2013","journal-title":"Semin Thromb Hemost"},{"issue":"4","key":"2024041115212086200_bib3","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1111\/j.1538-7836.2012.04653.x","article-title":"European Network of Rare Bleeding Disorders Group. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders","volume":"10","author":"Peyvandi","year":"2012","journal-title":"J\u00a0Thromb Haemost"},{"issue":"6","key":"2024041115212086200_bib4","doi-asserted-by":"crossref","first-page":"e488","DOI":"10.1111\/hae.13356","article-title":"High incidence of FXI deficiency in a Spanish town caused by 11 different mutations and the first duplication of F11: results from the Yecla study","volume":"23","author":"Esteban","year":"2017","journal-title":"Haemophilia"},{"issue":"6","key":"2024041115212086200_bib5","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1111\/j.1538-7836.2004.00723.x","article-title":"A\u00a0common ancestral mutation (C128X) occurring in 11 non-Jewish families from the UK with factor XI deficiency","volume":"2","author":"Bolton-Maggs","year":"2004","journal-title":"J\u00a0Thromb Haemost"},{"issue":"6","key":"2024041115212086200_bib6","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1182\/blood.V51.6.1223.1223","article-title":"High gene frequency of factor XI(PTA) deficiency in Ashkenazi Jews","volume":"51","author":"Seligsohn","year":"1978","journal-title":"Blood"},{"issue":"3","key":"2024041115212086200_bib7","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1056\/NEJM199107183250303","article-title":"Factor XI deficiency in Ashkenazi Jews in Israel","volume":"325","author":"Asakai","year":"1991","journal-title":"N\u00a0Engl J Med"},{"issue":"3","key":"2024041115212086200_bib8","doi-asserted-by":"crossref","first-page":"e2014810118","DOI":"10.1073\/pnas.2014810118","article-title":"Kallikrein directly interacts with and activates factor IX, resulting in thrombin generation and fibrin formation independent of factor XI","volume":"118","author":"Kearney","year":"2021","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"20","key":"2024041115212086200_bib9","doi-asserted-by":"crossref","first-page":"5025","DOI":"10.1182\/bloodadvances.2020002740","article-title":"Bleeding severity in patients with rare bleeding disorders: real-life data from the RBiN study","volume":"4","author":"Saes","year":"2020","journal-title":"Blood Adv"},{"issue":"15","key":"2024041115212086200_bib10","doi-asserted-by":"crossref","first-page":"1445","DOI":"10.1182\/blood.2023020719","article-title":"Biology of factor XI","volume":"143","author":"Moellmer","year":"2024","journal-title":"Blood"},{"issue":"1","key":"2024041115212086200_bib11","first-page":"9","article-title":"Age dependency for coagulation parameters in paediatric populaitons. Results of a multicentre study aimed at defining the age-specific reference ranges","volume":"116","author":"Toulon","year":"2016","journal-title":"Thromb Haemost"},{"issue":"1","key":"2024041115212086200_bib12","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/MBC.0000000000000660","article-title":"Intracranial hemorrhage in congenital bleeding disorders","volume":"29","author":"Tabibian","year":"2018","journal-title":"Blood Coagul Fibrinolysis"},{"issue":"5","key":"2024041115212086200_bib13","doi-asserted-by":"crossref","first-page":"e20173485","DOI":"10.1542\/peds.2017-3485","article-title":"Relevance of abusive head trauma to intracranial hemorrhages and bleeding disorders","volume":"141","author":"Anderst","year":"2018","journal-title":"Pediatrics"},{"key":"2024041115212086200_bib14","doi-asserted-by":"crossref","first-page":"102663","DOI":"10.1016\/j.bcmd.2022.102663","article-title":"Anti-TFPI for hemostasis induction in patients with rare bleeding disorders, an ex\u00a0vivo thrombin generation (TG) guided pilot study","volume":"95","author":"Barg","year":"2022","journal-title":"Blood Cells Mol Dis"},{"issue":"3","key":"2024041115212086200_bib15","doi-asserted-by":"crossref","first-page":"e756","DOI":"10.1542\/peds.2012-1990","article-title":"Male circumcision","volume":"130","author":"Blank","year":"2012","journal-title":"Pediatrics"},{"issue":"3","key":"2024041115212086200_bib16","doi-asserted-by":"crossref","first-page":"e29545","DOI":"10.1002\/pbc.29545","article-title":"Pediatric severe factor XI deficiency: a multicenter study","volume":"69","author":"Barg","year":"2022","journal-title":"Pediatr Blood Cancer"},{"issue":"3","key":"2024041115212086200_bib17","doi-asserted-by":"crossref","first-page":"e151","DOI":"10.1111\/hae.12577","article-title":"Haemorrhagic complications with adenotonsillectomy in children and young adults with bleeding disorders","volume":"21","author":"Warad","year":"2015","journal-title":"Haemophilia"},{"issue":"7","key":"2024041115212086200_bib18","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1080\/17474086.2020.1772745","article-title":"The clinical management of factor XI deficiency in pregnant women","volume":"13","author":"Wheeler","year":"2020","journal-title":"Expert Rev Hematol"},{"issue":"2","key":"2024041115212086200_bib19","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1111\/hae.12856","article-title":"Gynaecological and obstetrical bleeding in women with factor XI deficiency - a systematic review","volume":"22","author":"Wiewel-Verschueren","year":"2016","journal-title":"Haemophilia"},{"issue":"3","key":"2024041115212086200_bib20","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1111\/j.1471-0528.1998.tb10093.x","article-title":"Pregnancy in women with von Willebrand\u2019s disease or factor XI deficiency","volume":"105","author":"Kadir","year":"1998","journal-title":"Br J Obstet Gynaecol"},{"issue":"5","key":"2024041115212086200_bib21","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1111\/j.1471-0528.2007.01296.x","article-title":"Pregnancy outcome in factor XI deficiency: incidence of miscarriage, antenatal and postnatal haemorrhage in 33 women with factor XI deficiency","volume":"114","author":"Myers","year":"2007","journal-title":"BJOG"},{"issue":"3","key":"2024041115212086200_bib22","first-page":"140","article-title":"Longitudinal evaluation of markers of hemostasis in pregnancy","volume":"115","author":"Hammerova","year":"2014","journal-title":"Bratisl Lek Listy"},{"issue":"10","key":"2024041115212086200_bib23","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1080\/00016340903144238","article-title":"The obstetric experience of women with factor XI deficiency","volume":"88","author":"Chi","year":"2009","journal-title":"Acta Obstet Gynecol Scand"},{"issue":"1","key":"2024041115212086200_bib24","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1097\/00001721-200501000-00006","article-title":"Plasma replacement therapy during labor is not mandatory for women with severe factor XI deficiency","volume":"16","author":"Salomon","year":"2005","journal-title":"Blood Coagul Fibrinolysis"},{"issue":"4","key":"2024041115212086200_bib25","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1111\/hae.12628","article-title":"Bleeding phenotype and correlation with factor XI (FXI) activity in congenital FXI deficiency: results of a retrospective study from a single centre","volume":"21","author":"Santoro","year":"2015","journal-title":"Haemophilia"},{"key":"2024041115212086200_bib26","doi-asserted-by":"crossref","first-page":"1076029619880262","DOI":"10.1177\/1076029619880262","article-title":"Peri- and postpartum management of patients with factor XI deficiency","volume":"25","author":"Gerber","year":"2019","journal-title":"Clin Appl Thromb Hemost"},{"issue":"4","key":"2024041115212086200_bib27","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1111\/hae.14081","article-title":"Postpartum haemorrhage in women with mild factor XI deficiency","volume":"26","author":"Stoeckle","year":"2020","journal-title":"Haemophilia"},{"issue":"10","key":"2024041115212086200_bib28","doi-asserted-by":"crossref","first-page":"1967","DOI":"10.1182\/bloodadvances.2022008648","article-title":"Obstetric and peri-operative management of patients with factor XI deficiency: a retrospective observational study","volume":"7","author":"Handa","year":"2023","journal-title":"Blood Adv"},{"issue":"1","key":"2024041115212086200_bib29","first-page":"75","article-title":"Prostatectomy in factor XI deficiency","volume":"117","author":"Kaufman","year":"1977","journal-title":"J\u00a0Urol"},{"issue":"4","key":"2024041115212086200_bib30","first-page":"528","article-title":"Factor XI deficiency: detection and management during urological surgery","volume":"119","author":"Sidi","year":"1978","journal-title":"J\u00a0Urol"},{"issue":"9","key":"2024041115212086200_bib31","doi-asserted-by":"crossref","first-page":"3097","DOI":"10.1182\/blood-2007-06-096545","article-title":"Venous thrombosis in the elderly: more questions than answers","volume":"110","author":"Silverstein","year":"2007","journal-title":"Blood"},{"issue":"20","key":"2024041115212086200_bib32","doi-asserted-by":"crossref","first-page":"4083","DOI":"10.1182\/bloodadvances.2021005695","article-title":"Anticoagulant therapy in patients with congenital FXI deficiency","volume":"5","author":"Bravo-Perez","year":"2021","journal-title":"Blood Adv"},{"issue":"3","key":"2024041115212086200_bib33","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1182\/blood-2014-12-616565","article-title":"Sample conditions determine the ability of thrombin generation parameters to identify bleeding phenotype in FXI deficiency","volume":"126","author":"Pike","year":"2015","journal-title":"Blood"},{"issue":"4","key":"2024041115212086200_bib34","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1111\/j.1365-2516.2008.01663.x","article-title":"Recombinant factor VIIa to prevent surgical bleeding in factor XI deficiency","volume":"14","author":"O\u2019Connell","year":"2008","journal-title":"Haemophilia"},{"issue":"5","key":"2024041115212086200_bib35","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1111\/j.1365-2516.2009.02043.x","article-title":"Lower doses of rFVIIa therapy are safe and effective for surgical interventions in patients with severe FXI deficiency and inhibitors","volume":"15","author":"Kenet","year":"2009","journal-title":"Haemophilia"},{"issue":"10","key":"2024041115212086200_bib36","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1056\/NEJM200003093421004","article-title":"High levels of coagulation factor XI as a risk factor for venous thrombosis","volume":"342","author":"Meijers","year":"2000","journal-title":"N\u00a0Engl J Med"},{"issue":"5","key":"2024041115212086200_bib37","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1111\/jth.14415","article-title":"Factor XI and recurrent venous thrombosis: an observational cohort study","volume":"17","author":"Kyrle","year":"2019","journal-title":"J\u00a0Thromb Haemost"},{"issue":"2","key":"2024041115212086200_bib38","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1160\/TH10-05-0307","article-title":"Patients with severe factor XI deficiency have a reduced incidence of deep-vein thrombosis","volume":"105","author":"Salomon","year":"2011","journal-title":"Thromb Haemost"},{"issue":"4","key":"2024041115212086200_bib39","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1046\/j.1538-7836.2003.00195.x","article-title":"Inherited factor XI deficiency confers no protection against acute myocardial infarction","volume":"1","author":"Salomon","year":"2003","journal-title":"J\u00a0Thromb Haemost"},{"issue":"9","key":"2024041115212086200_bib40","doi-asserted-by":"crossref","first-page":"1210","DOI":"10.1182\/blood-2016-09-742262","article-title":"Factor XI deficiency is associated with lower risk for cardiovascular and venous thromboembolism events","volume":"129","author":"Preis","year":"2017","journal-title":"Blood"},{"issue":"15","key":"2024041115212086200_bib41","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1182\/blood.2023020722","article-title":"Targeting factor XI and factor XIa to prevent thrombosis","volume":"143","author":"Gailani","year":"2024","journal-title":"Blood"},{"issue":"3","key":"2024041115212086200_bib42","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1055\/s-0038-1657272","article-title":"Acquired factor XI deficiency in systemic lupus erythematosus","volume":"48","author":"Vercellotti","year":"1982","journal-title":"Thromb Haemost"},{"issue":"4","key":"2024041115212086200_bib43","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1136\/jcp.45.4.352","article-title":"Acquired factor XI inhibitor in chronic lymphocytic leukaemia","volume":"45","author":"Goodrick","year":"1992","journal-title":"J\u00a0Clin Pathol"},{"issue":"3","key":"2024041115212086200_bib44","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1001\/archinte.1984.00350150119031","article-title":"Spontaneous factor XI inhibitors: seven additional cases and a review of the literature","volume":"144","author":"Reece","year":"1984","journal-title":"Arch Intern Med"},{"issue":"4","key":"2024041115212086200_bib45","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1002\/ajh.2830260405","article-title":"Acquired factor XI inhibitors in congenitally deficient patients","volume":"26","author":"Schnall","year":"1987","journal-title":"Am J Hematol"},{"issue":"3","key":"2024041115212086200_bib46","doi-asserted-by":"crossref","first-page":"1306","DOI":"10.1182\/blood-2007-08-108449","article-title":"Induction of an inhibitor antibody to factor XI in a patient with severe inherited factor XI deficiency by Rh immune globulin","volume":"111","author":"Zucker","year":"2008","journal-title":"Blood"},{"issue":"1 suppl 1","key":"2024041115212086200_bib47","doi-asserted-by":"crossref","first-page":"S10","DOI":"10.1053\/j.seminhematol.2005.11.018","article-title":"Inhibitors to Factor XI in patients with severe factor XI deficiency","volume":"43","author":"Salomon","year":"2006","journal-title":"Semin Hematol"},{"issue":"1","key":"2024041115212086200_bib48","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1111\/j.1365-2141.1993.tb03044.x","article-title":"Successful childbirth by a patient with congenital factor XI deficiency and an acquired inhibitor","volume":"84","author":"Ginsberg","year":"1993","journal-title":"Br J Haematol"},{"issue":"23","key":"2024041115212086200_bib49","doi-asserted-by":"crossref","first-page":"2161","DOI":"10.1056\/NEJMoa2113194","article-title":"AXIOMATIC-TKR investigators. milvexian for the prevention of venous thromboembolism","volume":"385","author":"Weitz","year":"2021","journal-title":"N\u00a0Engl J Med"},{"issue":"10357","key":"2024041115212086200_bib50","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1016\/S0140-6736(22)01588-4","article-title":"Factor XIa inhibition with asundexian after acute non-cardioembolic ischaemic stroke (PACIFIC-Stroke): an international, randomised, double-blind, placebo-controlled, phase 2b trial","volume":"400","author":"Shoamanesh","year":"2022","journal-title":"Lancet"},{"key":"2024041115212086200_bib51","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.thromres.2016.11.008","article-title":"European Network of Rare Bleeding Disorders (EN-RBD) group. Establishment of a bleeding score as a diagnostic tool for patients with rare bleeding disorders","volume":"148","author":"Palla","year":"2016","journal-title":"Thromb Res"},{"issue":"1","key":"2024041115212086200_bib52","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1111\/jth.13883","article-title":"The ISTH Bleeding Assessment Tool and the risk of future bleeding","volume":"16","author":"Fasulo","year":"2018","journal-title":"J\u00a0Thromb Haemost"},{"issue":"8","key":"2024041115212086200_bib53","doi-asserted-by":"crossref","first-page":"3035","DOI":"10.1182\/blood.V86.8.3035.3035","article-title":"Feedback activation of factor XI by thrombin in plasma results in additional formation of thrombin that protects fibrin clots from fibrinolysis","volume":"86","author":"Von dem Borne","year":"1995","journal-title":"Blood"},{"issue":"5","key":"2024041115212086200_bib54","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1111\/j.1365-2516.2006.01304.x","article-title":"Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy","volume":"12","author":"Salomon","year":"2006","journal-title":"Haemophilia"},{"issue":"1","key":"2024041115212086200_bib55","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1055\/s-0038-1645682","article-title":"The effect of combined factor XI deficiency with von Willebrand factor abnormalities on haemorrhagic diathesis","volume":"63","author":"Tavori","year":"1990","journal-title":"Thromb Haemost"},{"issue":"2","key":"2024041115212086200_bib56","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.thromres.2015.06.025","article-title":"The impact of thrombin generation and rotation thromboelastometry on assessment of severity of factor XI deficiency","volume":"136","author":"Livnat","year":"2015","journal-title":"Thromb Res"},{"issue":"5","key":"2024041115212086200_bib57","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1111\/j.1365-2516.2010.02246.x","article-title":"Thrombin generation in patients with factor XI deficiency and clinical bleeding risk","volume":"16","author":"Rugeri","year":"2010","journal-title":"Haemophilia"},{"key":"2024041115212086200_bib58","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1016\/j.cca.2021.10.021","article-title":"Evaluation of thrombin generation assay in factor XI deficiency","volume":"523","author":"Kasonga","year":"2021","journal-title":"Clin Chim Acta"},{"issue":"3","key":"2024041115212086200_bib59","doi-asserted-by":"crossref","first-page":"e273","DOI":"10.1055\/s-0039-1693485","article-title":"Impact of deficiency of intrinsic coagulation factors XI and XII on ex\u00a0vivo thrombus formation and clot lysis","volume":"3","author":"Govers-Riemslag","year":"2019","journal-title":"TH Open"},{"issue":"2","key":"2024041115212086200_bib60","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1111\/hae.14506","article-title":"Report of surgeries, their outcome and the thrombin generation assay in patients with factor XI deficiency: a retrospective single-centre study","volume":"28","author":"D\u00e9sage","year":"2022","journal-title":"Haemophilia"},{"issue":"12","key":"2024041115212086200_bib61","doi-asserted-by":"crossref","first-page":"1927","DOI":"10.1055\/s-0039-1696685","article-title":"Single low dose of rFVIIa combined with antifibrinolytic agent is a simple and safe treatment for factor XI-deficient patients undergoing surgery","volume":"119","author":"Salomon","year":"2019","journal-title":"Thromb Haemost"},{"issue":"2","key":"2024041115212086200_bib62","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1111\/hae.13112","article-title":"Evaluation of the use of global haemostasis assays to monitor treatment in factor XI deficiency","volume":"23","author":"Pike","year":"2017","journal-title":"Haemophilia"},{"issue":"3","key":"2024041115212086200_bib63","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1111\/hae.12846","article-title":"In\u00a0vitro comparison of the effect of two factor XI (FXI) concentrates on thrombin generation in major FXI deficiency","volume":"22","author":"Pike","year":"2016","journal-title":"Haemophilia"},{"issue":"7","key":"2024041115212086200_bib64","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1111\/jth.12600","article-title":"Abnormal plasma clot structure and stability distinguish bleeding risk in patients with severe factor XI deficiency","volume":"12","author":"Zucker","year":"2014","journal-title":"J\u00a0Thromb Haemost"},{"issue":"10","key":"2024041115212086200_bib65","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1182\/bloodadvances.2017015123","article-title":"Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency","volume":"2","author":"Gidley","year":"2018","journal-title":"Blood Adv"},{"issue":"1","key":"2024041115212086200_bib66","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1182\/hematology.2022000397","article-title":"Long-term prophylaxis: what are our options and how to define success?","volume":"2022","author":"Manco-Johnson","year":"2022","journal-title":"Hematology Am Soc Hematol Educ Program"},{"issue":"5","key":"2024041115212086200_bib67","doi-asserted-by":"crossref","first-page":"e12546","DOI":"10.1002\/rth2.12546","article-title":"Tranexamic acid evidence and controversies: an illustrated review","volume":"5","author":"Relke","year":"2021","journal-title":"Res Pract Thromb Haemost"},{"issue":"3","key":"2024041115212086200_bib68","doi-asserted-by":"crossref","first-page":"238","DOI":"10.4103\/UA.UA_41_20","article-title":"Efficacy of tranexamic acid in decreasing primary hemorrhage in transurethral resection of the prostate: a novel combination of intravenous and topical\u00a0approach","volume":"13","author":"Gupta","year":"2021","journal-title":"Urol Ann"},{"issue":"2","key":"2024041115212086200_bib69","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1053\/j.seminhematol.2020.07.006","article-title":"Recent advances in use of fresh frozen plasma, cryoprecipitate, immunoglobulins, and clotting factors for transfusion support in patients with hematologic disease","volume":"57","author":"Nair","year":"2020","journal-title":"Semin Hematol"},{"issue":"1","key":"2024041115212086200_bib70","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.beha.2005.01.033","article-title":"Risks and side effects of therapy with plasma and plasma fractions","volume":"19","author":"MacLennan","year":"2006","journal-title":"Best Pract Res Clin Haematol"},{"issue":"suppl 1","key":"2024041115212086200_bib71","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1016\/S0049-3848(02)00149-4","article-title":"Fresh frozen plasma in the pediatric age group and in congenital coagulation factor deficiency","volume":"107","author":"Muntean","year":"2002","journal-title":"Thromb Res"},{"issue":"03","key":"2024041115212086200_bib72","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1055\/s-0038-1648439","article-title":"Production and therapeutic use of a factor XI concentrate from plasma","volume":"67","author":"Bolton-Maggs","year":"1992","journal-title":"Thromb Haemost"},{"issue":"4","key":"2024041115212086200_bib73","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1111\/hae.12655","article-title":"Factor XI replacement for inherited factor XI deficiency in routine\u00a0clinical practice: results of the HEMOLEVEN prospective 3-year postmarketing study","volume":"21","author":"Bauduer","year":"2015","journal-title":"Haemophilia"},{"issue":"4","key":"2024041115212086200_bib74","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1111\/hae.12682","article-title":"Ongoing risk of thrombosis with factor XI concentrate: 5 years experience in two centres","volume":"21","author":"Batty","year":"2015","journal-title":"Haemophilia"},{"issue":"3","key":"2024041115212086200_bib75","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1111\/hae.12868","article-title":"Safety and efficacy of factor XI (FXI) concentrate use in patients with FXI deficiency: a single-centre experience of 19\u00a0years","volume":"22","author":"Ling","year":"2016","journal-title":"Haemophilia"},{"issue":"4","key":"2024041115212086200_bib76","doi-asserted-by":"crossref","first-page":"e349","DOI":"10.1111\/hae.12457","article-title":"FXI concentrate use and risk of thrombosis","volume":"20","author":"Bolton-Maggs","year":"2014","journal-title":"Haemophilia"},{"issue":"1","key":"2024041115212086200_bib77","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1111\/j.1365-2516.2005.01052.x","article-title":"Treatment of factor XI inhibitor using recombinant activated factor VIIa","volume":"11","author":"Bern","year":"2005","journal-title":"Haemophilia"},{"issue":"3","key":"2024041115212086200_bib78","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1160\/TH10-12-0816","article-title":"Monitoring low dose recombinant factor VIIa therapy in patients with severe factor XI deficiency undergoing surgery","volume":"106","author":"Riddell","year":"2011","journal-title":"Thromb Haemost"},{"issue":"6","key":"2024041115212086200_bib79","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1111\/j.1365-2516.2011.02525.x","article-title":"The role of recombinant factor VIIa for obstetric block in women with severe factor XI deficiency","volume":"17","author":"Setty","year":"2011","journal-title":"Haemophilia"},{"issue":"6","key":"2024041115212086200_bib80","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1179\/1607845412Y.0000000027","article-title":"Recombinant FVIIa concentrate-associated thrombotic events in congenital bleeding disorders other than hemophilias","volume":"17","author":"Girolami","year":"2012","journal-title":"Hematology"},{"issue":"3","key":"2024041115212086200_bib81","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"},{"issue":"6","key":"2024041115212086200_bib82","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1016\/j.hoc.2021.07.012","article-title":"Factor XI deficiency","volume":"35","author":"Lewandowska","year":"2021","journal-title":"Hematol Oncol Clin North Am"},{"issue":"8","key":"2024041115212086200_bib83","first-page":"e193","article-title":"Management of inherited bleeding disorders in pregnancy: green-top guideline no. 71 (joint with UKHCDO)","volume":"124","author":"Pavord","year":"2017","journal-title":"BJOG"},{"issue":"4","key":"2024041115212086200_bib84","doi-asserted-by":"crossref","first-page":"e12491","DOI":"10.1002\/rth2.12491","article-title":"Evaluating hemostatic thresholds for neuraxial anesthesia in adults with hemorrhagic disorders and tendencies: a scoping review","volume":"5","author":"Peterson","year":"2021","journal-title":"Res Pract Thromb Haemost"},{"issue":"5","key":"2024041115212086200_bib85","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1111\/hae.14079","article-title":"Epidural\/spinal anesthesia during delivery in women with factor XI deficiency, a single center experience","volume":"26","author":"Shacham Abulafia","year":"2020","journal-title":"Haemophilia"},{"issue":"1","key":"2024041115212086200_bib86","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1111\/bjh.18379","article-title":"Current and future therapies for haemophilia\u2014beyond factor replacement therapies","volume":"200","author":"Nogami","year":"2023","journal-title":"Br J Haematol"},{"issue":"9","key":"2024041115212086200_bib87","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1056\/NEJMoa1616569","article-title":"Targeting of antithrombin in hemophilia A or B with RNAi therapy","volume":"377","author":"Pasi","year":"2017","journal-title":"N\u00a0Engl J Med"},{"issue":"10386","key":"2024041115212086200_bib88","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1016\/S0140-6736(23)00284-2","article-title":"Efficacy and safety of fitusiran prophylaxis in people with haemophilia A or haemophilia B with inhibitors (ATLAS-INH): a multicentre, open-label, randomised phase 3 trial","volume":"401","author":"Young","year":"2023","journal-title":"Lancet"},{"issue":"11","key":"2024041115212086200_bib89","doi-asserted-by":"crossref","first-page":"3422","DOI":"10.1182\/bloodadvances.2021006403","article-title":"Long-term efficacy and safety of subcutaneous concizumab prophylaxis in hemophilia A and hemophilia A\/B with inhibitors","volume":"6","author":"Shapiro","year":"2022","journal-title":"Blood Adv"},{"issue":"2","key":"2024041115212086200_bib90","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1111\/bjh.18495","article-title":"Long-term safety and efficacy of the anti-tissue factor pathway inhibitor marstacimab in participants with\u00a0severe haemophilia: phase II study results","volume":"200","author":"Mahlangu","year":"2023","journal-title":"Br J Haematol"},{"issue":"3","key":"2024041115212086200_bib91","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1016\/j.jtha.2022.10.005","article-title":"Tissue factor pathway inhibitor is a potential modifier of bleeding risk in factor XI deficiency","volume":"21","author":"Reitsma","year":"2023","journal-title":"J\u00a0Thromb Haemost"}],"container-title":["Blood"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ashpublications.org\/blood\/article-pdf\/143\/15\/1455\/2221594\/blood_bld-2023-020721-c-main.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/ashpublications.org\/blood\/article-pdf\/143\/15\/1455\/2221594\/blood_bld-2023-020721-c-main.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,11]],"date-time":"2024-04-11T11:22:07Z","timestamp":1712834527000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/blood\/article\/143\/15\/1455\/507070\/Factor-XI-deficiency-phenotypic-age-related"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,11]]},"references-count":91,"journal-issue":{"issue":"15","published-print":{"date-parts":[[2024,4,11]]}},"URL":"https:\/\/doi.org\/10.1182\/blood.2023020721","relation":{},"ISSN":["0006-4971","1528-0020"],"issn-type":[{"value":"0006-4971","type":"print"},{"value":"1528-0020","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,11]]}}}