{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T12:15:13Z","timestamp":1789820113855,"version":"4.0.1"},"reference-count":77,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"abstract":"<jats:sec>\n                    <jats:title>ABSTRACT<\/jats:title>\n                    <jats:p>Rare bleeding disorders (RBDs) represent a diverse group of inherited conditions involving coagulation factors or platelets. These conditions, such as Glanzmann thrombasthenia (GT) or severe coagulation factor deficiencies, are uncommon. In contrast, bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion without an identifiable genetic defect and has emerged as the most frequent finding in patients evaluated for a lifelong bleeding tendency. Their clinical presentation is highly variable and often not well predicted by standard laboratory findings. This State\u2010of\u2010the\u2010Art review summarizes current understanding and recent developments across four representative conditions: inherited platelet function disorders (IPFDs) highlighting GT, fibrinogen disorders, factor XI (FXI) deficiency, and BDUC. In IPFDs, management remains challenging because of wide variability in bleeding severity and the need for specialized expertise. In fibrinogen disorders, the limited assay availability hampers consistent classification and management. FXI deficiency illustrates the weak association between factor levels and bleeding phenotype, underlining the importance of individualized care. Finally, studies on BDUC reveal overlaps with mild inherited disorders and highlight the value of global haemostatic and fibrinolytic assays in improving diagnostic precision. Across these four disorders, common priorities emerge, such as harmonizing diagnostic approaches, strengthening international registries, and integrating patient\u2010reported outcomes to better capture real\u2010life impact.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70278","type":"journal-article","created":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T11:04:43Z","timestamp":1776337483000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Rare Bleeding Disorders and Bleeding Disorder of Unknown Cause: Current Understanding and Recent Developments"],"prefix":"10.1111","author":[{"given":"Alessandro","family":"Casini","sequence":"first","affiliation":[{"name":"Division of Angiology and Hemostasis University Hospitals of Geneva and Faculty of Medicine of Geneva  Geneva Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Klamroth","sequence":"additional","affiliation":[{"name":"Department For Internal Medicine Angiology and Haemostaseology Vivantes Hospital in Friedrichshain  Berlin Germany"},{"name":"Institute of Experimental Hematology and Transfusion Medicine, Medical Faculty University Hospital Bonn, University of Bonn  Bonn Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bauke","family":"Haisma","sequence":"additional","affiliation":[{"name":"Hemophilia Treatment Centre Nijmegen\u2010Eindhoven\u2010Maastricht, Department of Hematology Radboud University Medical Centre Nijmegen  Nijmegen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dino","family":"Mehic","sequence":"additional","affiliation":[{"name":"Division of Hematology and Hemostaseology, Department of Medicine I Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saskia E. M.","family":"Schols","sequence":"additional","affiliation":[{"name":"Hemophilia Treatment Centre Nijmegen\u2010Eindhoven\u2010Maastricht, Department of Hematology Radboud University Medical Centre Nijmegen  Nijmegen Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,4,16]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"crossref","DOI":"10.1016\/j.rpth.2024.102460","article-title":"Rare Inherited Coagulation Disorders: No Longer Orphan and Neglected","volume":"8","author":"Mohsenian S.","year":"2024","journal-title":"Research Practices in Thrombosis and Haemostasis"},{"issue":"3","key":"e_1_2_9_3_1","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1111\/hae.14986","article-title":"Rare Bleeding Disorders: Advances in Management","volume":"30","author":"Casini A.","year":"2024","journal-title":"Haemophilia"},{"key":"e_1_2_9_4_1","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1111\/j.1365-2141.2006.06343.x","article-title":"A Review of Inherited Platelet Disorders With Guidelines for Their Management on Behalf of the UKHCDO","volume":"135","author":"Bolton\u2010Maggs P. 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