{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T00:10:17Z","timestamp":1792627817926,"version":"4.5.0"},"reference-count":43,"publisher":"Elsevier BV","issue":"7","license":[{"start":{"date-parts":[[2024,10,1]],"date-time":"2024-10-01T00:00:00Z","timestamp":1727740800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,10,1]],"date-time":"2024-10-01T00:00:00Z","timestamp":1727740800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,10,17]],"date-time":"2024-10-17T00:00:00Z","timestamp":1729123200000},"content-version":"vor","delay-in-days":16,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["rpthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Research and Practice in Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2024,10]]},"DOI":"10.1016\/j.rpth.2024.102604","type":"journal-article","created":{"date-parts":[[2024,10,23]],"date-time":"2024-10-23T16:03:15Z","timestamp":1729699395000},"page":"102604","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"title":["Enhanced thrombin and plasmin generation profiles in alpha-2-antiplasmin\u2013deficient patients: Data from the Rare Bleeding disorders in the Netherlands study"],"prefix":"10.1016","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-5149-8864","authenticated-orcid":false,"given":"Bauke","family":"Haisma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanna R.","family":"Rijpma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjon H.","family":"Cnossen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul L.","family":"den Exter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilmar C.","family":"Kruis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karina","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurens","family":"Nieuwenhuizen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nick","family":"van Es","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger E.G.","family":"Schutgens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole M.A.","family":"Blijlevens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Waander L.","family":"van Heerde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2423-2829","authenticated-orcid":false,"given":"Saskia E.M.","family":"Schols","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.rpth.2024.102604_bib1","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1111\/j.1365-2516.2008.01766.x","article-title":"Alpha2-antiplasmin and its deficiency: fibrinolysis out of balance","volume":"14","author":"Carpenter","year":"2008","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2024.102604_bib2","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/0009-8981(78)90481-3","article-title":"The alpha2-plasmin inhibitor levels in liver diseases","volume":"84","author":"Aoki","year":"1978","journal-title":"Clin Chim Acta"},{"key":"10.1016\/j.rpth.2024.102604_bib3","doi-asserted-by":"crossref","first-page":"2478","DOI":"10.1172\/JCI118694","article-title":"The kidney is a major site of alpha(2)-antiplasmin production","volume":"97","author":"Menoud","year":"1996","journal-title":"J\u00a0Clin Invest"},{"key":"10.1016\/j.rpth.2024.102604_bib4","doi-asserted-by":"crossref","first-page":"1498","DOI":"10.1111\/jth.14160","article-title":"Hemorrhagic disorders of fibrinolysis: a clinical review","volume":"16","author":"Saes","year":"2018","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib5","doi-asserted-by":"crossref","DOI":"10.7754\/Clin.Lab.2020.200207","article-title":"Congenital alpha-2 antiplasmin deficiency: a literature survey and analysis of 123 cases","volume":"66","author":"Matrane","year":"2020","journal-title":"Clin Lab"},{"key":"10.1016\/j.rpth.2024.102604_bib6","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1046\/j.1365-2141.2001.02845.x","article-title":"Congenital alpha(2)-plasmin inhibitor deficiencies: a review","volume":"114","author":"Favier","year":"2001","journal-title":"Br J Haematol"},{"key":"10.1016\/j.rpth.2024.102604_bib7","doi-asserted-by":"crossref","first-page":"978","DOI":"10.1007\/s002470050513","article-title":"A\u00a0case of intramedullary haematoma associated with congenital alpha2-plasmin inhibitor deficiency","volume":"28","author":"Devaussuzenet","year":"1998","journal-title":"Pediatr Radiol"},{"key":"10.1016\/j.rpth.2024.102604_bib8","first-page":"321","article-title":"Intramedullary multiple hematomas in siblings with congenital alpha-2-plasmin inhibitor deficiency: orthopedic surgery with protection by tranexamic acid","volume":"21","author":"Takahashi","year":"1991","journal-title":"Haemostasis"},{"key":"10.1016\/j.rpth.2024.102604_bib9","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1172\/JCI109387","article-title":"Congenital deficiency of alpha 2-plasmin inhibitor associated with severe hemorrhagic tendency","volume":"63","author":"Aoki","year":"1979","journal-title":"J\u00a0Clin Invest"},{"key":"10.1016\/j.rpth.2024.102604_bib10","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/S0140-6736(79)91481-8","article-title":"Homozygous alpha 2-antiplasmin deficiency","volume":"2","author":"Kluft","year":"1979","journal-title":"Lancet"},{"key":"10.1016\/j.rpth.2024.102604_bib11","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1016\/S0140-6736(78)91973-6","article-title":"Alpha2-plasmin-inhibitor deficiency (Miyasato disease)","volume":"2","author":"Koie","year":"1978","journal-title":"Lancet"},{"key":"10.1016\/j.rpth.2024.102604_bib12","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1016\/0049-3848(85)90302-0","article-title":"Severe hemorrhagic tendency in heterozygous alpha 2-antiplasmin deficiency","volume":"40","author":"Kordich","year":"1985","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2024.102604_bib13","first-page":"328","article-title":"Alpha 2-antiplasmin deficiency. An overlooked cause of hemorrhage","volume":"15","author":"Griffin","year":"1993","journal-title":"Am J Pediatr Hematol Oncol"},{"key":"10.1016\/j.rpth.2024.102604_bib14","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1111\/j.1538-7836.2005.01114.x","article-title":"Bleeding and thrombotic problems in a patient with alpha2 plasmin inhibitor deficiency","volume":"3","author":"Hayward","year":"2005","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib15","doi-asserted-by":"crossref","DOI":"10.1155\/2011\/698648","article-title":"Alpha II antiplasmin deficiency complicating pregnancy: a case report","volume":"2011","author":"Dawley","year":"2011","journal-title":"Obstet Gynecol Int"},{"key":"10.1016\/j.rpth.2024.102604_bib16","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1055\/s-0038-1646773","article-title":"Mild haemostatic problems associated with congenital heterozygous alpha 2-antiplasmin deficiency","volume":"59","author":"Leebeek","year":"1988","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib17","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1111\/j.1365-2516.2008.01894.x","article-title":"Menorrhagia and reproductive health in rare bleeding disorders: a study from the Indian subcontinent","volume":"15","author":"Vijapurkar","year":"2009","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2024.102604_bib18","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1046\/j.1365-2141.1999.01708.x","article-title":"A\u00a0novel missense mutation in the human plasmin inhibitor (alpha2-antiplasmin) gene associated with a bleeding tendency","volume":"107","author":"Lind","year":"1999","journal-title":"Br J Haematol"},{"key":"10.1016\/j.rpth.2024.102604_bib19","doi-asserted-by":"crossref","DOI":"10.1002\/rth2.12681","article-title":"Fibrinolytic assays in bleeding of unknown cause: Improvement in diagnostic yield","volume":"6","author":"Valke","year":"2022","journal-title":"Res Pract Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib20","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"},{"key":"10.1016\/j.rpth.2024.102604_bib21","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1179\/102453311X13085644680348","article-title":"A\u00a0novel hemostasis assay for the simultaneous measurement of coagulation and fibrinolysis","volume":"16","author":"van Geffen","year":"2011","journal-title":"Hematology"},{"key":"10.1016\/j.rpth.2024.102604_bib22","doi-asserted-by":"crossref","DOI":"10.1016\/j.rpth.2023.100081","article-title":"Fibrinolysis: an illustrated review","volume":"7","author":"Risman","year":"2023","journal-title":"Res Pract Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib23","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/0049-3848(83)90244-X","article-title":"A\u00a0comparison of the abilities of plasma kallikrein, beta-factor XIIa, Factor XIa and urokinase to activate plasminogen","volume":"29","author":"Miles","year":"1983","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2024.102604_bib24","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1016\/j.thromres.2015.06.028","article-title":"The role of activated coagulation factor XII in overall clot stability and fibrinolysis","volume":"136","author":"Konings","year":"2015","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2024.102604_bib25","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1515\/hsz-2016-0329","article-title":"Structural and functional insight into pan-endopeptidase inhibition by \u03b12-macroglobulins","volume":"398","author":"Goulas","year":"2017","journal-title":"Biol Chem"},{"key":"10.1016\/j.rpth.2024.102604_bib26","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1111\/jth.15647","article-title":"Alpha-2-macroglobulin in hemostasis and thrombosis: an underestimated old double-edged sword","volume":"20","author":"Lagrange","year":"2022","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib27","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1111\/j.1538-7836.2007.02430.x","article-title":"TAFIa, PAI-1 and alpha-antiplasmin: complementary roles in regulating lysis of thrombi and plasma clots","volume":"5","author":"Mutch","year":"2007","journal-title":"J\u00a0Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib28","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1084\/jem.146.4.1033","article-title":"Plasmin inhibitor interactions. The effectiveness of alpha2-plasmin inhibitor in the presence of alpha2-macroglobulin","volume":"146","author":"Harpel","year":"1977","journal-title":"J\u00a0Exp Med"},{"key":"10.1016\/j.rpth.2024.102604_bib29","series-title":"Disorders of hemostasis and thrombosis: a clinical guide","author":"Goodnight","year":"2000"},{"key":"10.1016\/j.rpth.2024.102604_bib30","series-title":"Hemostasis and thrombosis: basic principles and clinical practice","author":"Marder","year":"2012"},{"key":"10.1016\/j.rpth.2024.102604_bib31","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1111\/hae.13842","article-title":"Thrombin and plasmin generation in patients with plasminogen or plasminogen activator inhibitor type 1 deficiency","volume":"25","author":"Saes","year":"2019","journal-title":"Haemophilia"},{"key":"10.1016\/j.rpth.2024.102604_bib32","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/j.thromres.2010.12.011","article-title":"Simultaneous thrombin and plasmin generation capacities in normal and abnormal states of coagulation and fibrinolysis in children and adults","volume":"127","author":"Simpson","year":"2011","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2024.102604_bib33","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1182\/blood.V73.1.185.185","article-title":"Activation\/inactivation of human factor V by plasmin","volume":"73","author":"Lee","year":"1989","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2024.102604_bib34","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/0049-3848(74)90119-4","article-title":"Activation of purified plasma factor VII by human plasmin, plasma kallikrein, and activated components of the human intrinsic blood coagulation system","volume":"5","author":"Laake","year":"1974","journal-title":"Thromb Res"},{"key":"10.1016\/j.rpth.2024.102604_bib35","doi-asserted-by":"crossref","first-page":"1649","DOI":"10.1182\/blood.V67.6.1649.1649","article-title":"Platelets modulate the proteolysis of factor VIII:C protein by plasmin","volume":"67","author":"Rick","year":"1986","journal-title":"Blood"},{"key":"10.1016\/j.rpth.2024.102604_bib36","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1055\/s-0038-1661226","article-title":"Effects of plasmin on rabbit platelets","volume":"53","author":"Guccione","year":"1985","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib37","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1097\/TA.0000000000003723","article-title":"Plasma-based assays distinguish hyperfibrinolysis and shutdown subgroups in trauma-induced coagulopathy","volume":"93","author":"Lawson","year":"2022","journal-title":"J\u00a0Trauma Acute Care Surg"},{"key":"10.1016\/j.rpth.2024.102604_bib38","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1161\/01.CIR.85.3.928","article-title":"Plasma fibrinopeptide A levels in patients with acute myocardial infarction treated with alteplase. Correlation with concomitant heparin, coronary artery patency, and recurrent ischemia. The European Cooperative Study Group","volume":"85","author":"Rapold","year":"1992","journal-title":"Circulation"},{"key":"10.1016\/j.rpth.2024.102604_bib39","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/0735-1097(94)00360-3","article-title":"Thrombin generation and activity during thrombolysis and concomitant heparin therapy in patients with acute myocardial infarction","volume":"25","author":"Merlini","year":"1995","journal-title":"J\u00a0Am Coll Cardiol"},{"key":"10.1016\/j.rpth.2024.102604_bib40","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1161\/01.CIR.83.3.937","article-title":"Increased thrombin levels during thrombolytic therapy in acute myocardial infarction. Relevance for the success of therapy","volume":"83","author":"Gulba","year":"1991","journal-title":"Circulation"},{"key":"10.1016\/j.rpth.2024.102604_bib41","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1161\/01.CIR.91.1.28","article-title":"Plasmin-mediated activation of contact system in response to pharmacological thrombolysis","volume":"91","author":"Ewald","year":"1995","journal-title":"Circulation"},{"key":"10.1016\/j.rpth.2024.102604_bib42","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1055\/s-0038-1647450","article-title":"Quantification of functional and inactivated alpha 2-macroglobulin in sepsis","volume":"65","author":"Abbink","year":"1991","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.rpth.2024.102604_bib43","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/0022-1759(90)90006-H","article-title":"Measurement of the \u2018fast' or complexed form of alpha 2 macroglobulin in biological fluids using a sandwich enzyme immunoassay","volume":"126","author":"Banks","year":"1990","journal-title":"J\u00a0Immunol Methods"}],"container-title":["Research and Practice in Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037924002991?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2475037924002991?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2024,11,22]],"date-time":"2024-11-22T00:23:27Z","timestamp":1732235007000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2475037924002991"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10]]},"references-count":43,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2024,10]]}},"alternative-id":["S2475037924002991"],"URL":"https:\/\/doi.org\/10.1016\/j.rpth.2024.102604","relation":{},"ISSN":["2475-0379"],"issn-type":[{"value":"2475-0379","type":"print"}],"subject":[],"published":{"date-parts":[[2024,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Enhanced thrombin and plasmin generation profiles in alpha-2-antiplasmin\u2013deficient patients: Data from the Rare Bleeding disorders in the Netherlands study","name":"articletitle","label":"Article Title"},{"value":"Research and Practice in Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.rpth.2024.102604","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Author(s). Published by Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.","name":"copyright","label":"Copyright"}],"article-number":"102604"}}