{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,1]],"date-time":"2026-02-01T12:03:12Z","timestamp":1769947392396,"version":"3.49.0"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2017,5,17]],"date-time":"2017-05-17T00:00:00Z","timestamp":1494979200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["FS\/11\/2\/28579"],"award-info":[{"award-number":["FS\/11\/2\/28579"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000882","name":"University of Aberdeen","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000882","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Acquired factor XIII (FXIII) deficiency is a rare and life\u2010threatening condition that is often misdiagnosed or missed completely. A 72\u2010year\u2010old woman presented with symptoms of major unprovoked bleeding but routine coagulation screening tests and platelet count were normal. Low activated FXIII (FXIIIa) activity levels and abnormal urea clot stability led to diagnosis of acquired FXIII deficiency. A modified Bethesda inhibitor titre of 1.6 Bethesda units\/ml indicated the presence of a FXIII inhibitor. Bleeding responded to a single dose of FXIII concentrate and immunosuppression with prednisolone induced remission. A subsequent relapse was treated with combined prednisolone and Rituximab resulting in a prolonged, ongoing remission. Here we analyse the mechanisms underlying this idiopathic case of acquired FXIII deficiency. Prospective analysis of patient plasma revealed minimal FXIIIa activity and antigen in presentation and relapse samples. Thrombi formed from these samples lysed rapidly and showed an absence of cross\u2010linked \u03b1<jats:sub>2<\/jats:sub>AP. Western blotting revealed the presence of FXIII\u2010B, indicating only FXIII\u2010A and FXIII\u2010A<jats:sub>2<\/jats:sub>B<jats:sub>2<\/jats:sub> were affected. FXIII activity and antigen levels normalised on remission. Our data suggest the presence of inhibitor\u2010induced clearance of FXIII from plasma. As a consequence, reduced thrombus stability was evident due to defective \u03b1<jats:sub>2<\/jats:sub>AP cross\u2010linking, thereby explaining symptoms of excessive bleeding.<\/jats:p>","DOI":"10.1111\/bjh.14759","type":"journal-article","created":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T04:43:04Z","timestamp":1495082584000},"page":"794-799","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Defective \u03b1<sub>2<\/sub>antiplasmin cross\u2010linking and thrombus stability in a case of acquired factor XIII deficiency"],"prefix":"10.1111","volume":"178","author":[{"given":"Joanne L.","family":"Mitchell","sequence":"first","affiliation":[{"name":"Institute of Medical Sciences University of Aberdeen Aberdeen UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonja","family":"Wright","sequence":"additional","affiliation":[{"name":"Department of Haematology Aberdeen Royal Infirmary Aberdeen UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sajida","family":"Kazi","sequence":"additional","affiliation":[{"name":"Department of Haematology Aberdeen Royal Infirmary Aberdeen UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henry G.","family":"Watson","sequence":"additional","affiliation":[{"name":"Department of Haematology Aberdeen Royal Infirmary Aberdeen UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7452-0813","authenticated-orcid":false,"given":"Nicola J.","family":"Mutch","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences University of Aberdeen Aberdeen UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI75386"},{"key":"e_1_2_8_3_1","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1182\/blood.V40.1.11.11","article-title":"Congenital deficiency of fibrin\u2010stabilizing factor (factor XIII): a report of four cases (two families) and family members","volume":"40","author":"Aziz M.A.","year":"1972","journal-title":"Blood"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1468-0734.2001.00049.x"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1988.tb02490.x"},{"key":"e_1_2_8_6_1","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1182\/blood.V28.1.34.34","article-title":"Factor 13 deficiency with severe hemorrhagic diathesis","volume":"28","author":"Fisher S.","year":"1966","journal-title":"Blood"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-333203"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(83)90081-6"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1080\/08998280.2006.11928166"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2012.02763.x"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12677"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2011.04315.x"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-9861(64)90235-8"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(65)90233-3"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-06-583070"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/0301-4622(74)80037-2"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.13058"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03963.x"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI109671"},{"key":"e_1_2_8_20_1","first-page":"1395","article-title":"Human Factor XIII from plasma and platelets. Molecular weights, subunit structures, proteolytic activation, and cross\u2010linking of fibrinogen and fibrin","volume":"248","author":"Schwartz M.L.","year":"1973","journal-title":"Journal of Biological Chemistry"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI110885"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.12877"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fbjh.14759","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.14759","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T17:07:57Z","timestamp":1696352877000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.14759"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,17]]},"references-count":21,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2017,9]]}},"alternative-id":["10.1111\/bjh.14759"],"URL":"https:\/\/doi.org\/10.1111\/bjh.14759","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,17]]},"assertion":[{"value":"2017-01-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-03-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-05-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}