{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T17:57:52Z","timestamp":1777485472522,"version":"3.51.4"},"reference-count":19,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,7,13]],"date-time":"2017-07-13T00:00:00Z","timestamp":1499904000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:sec><jats:title>BACKGROUND<\/jats:title><jats:p>Factor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors.<\/jats:p><\/jats:sec><jats:sec><jats:title>CASE REPORT<\/jats:title><jats:p>A 17\u2010year\u2010old female with systemic lupus erythematosus and end\u2010stage kidney disease secondary to Class IV lupus nephritis developed spontaneous subcutaneous and muscular hematomas and delayed major bleeding after invasive procedures. She had abnormal kaolin thromboelastography (kTEG; decreased maximal amplitude, representative of clot strength) initially attributed to thrombocytopenia and uremic platelet dysfunction, but her FXIII activity was undetectable, and a high\u2010titer antibody against FXIII was identified. She had improvement in clinical bleeding and in kaolin thromboelastogram result and transient improvement in FXIII activity after each dose of plasma\u2010derived FXIII concentrate (Corifact) or cryoprecipitate. Her inhibitor titers gradually improved with multiple immunosuppressive therapies and plasma exchange. While her FXIII activity level remained mildly decreased, she has not had additional significant bleeding.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSION<\/jats:title><jats:p>Treatment with either plasma\u2010derived FXIII or cryoprecipitate is an effective treatment to normalize the kTEG variables and clinical bleeding diatheses associated with acquired FXIII inhibitors. Higher doses may be needed in patients with high\u2010titer inhibitor.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.14185","type":"journal-article","created":{"date-parts":[[2017,7,14]],"date-time":"2017-07-14T04:16:41Z","timestamp":1500005801000},"page":"2159-2163","source":"Crossref","is-referenced-by-count":4,"title":["Treatment of an acquired Factor XIII inhibitor in an adolescent with systemic lupus erythematosus and renal failure"],"prefix":"10.1111","volume":"57","author":[{"given":"Cara A.","family":"Rabik","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Hematology"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meredith A.","family":"Atkinson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Nephrology"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sangeeta","family":"Sule","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Rheumatology Johns Hopkins University Baltimore Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John J.","family":"Strouse","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Hematology; and the Department of Pediatrics, Division of Pediatric Hematology\/Oncology Duke University Durham North Carolina."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,7,13]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.5482\/HAMO-13-08-0046"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-012-0818-3"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12145"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2008-09-179333"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1613334"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.4084\/mjhid.2016.037"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0b013e328335d0e4"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02459.x"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(01)00395-4"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.12481"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH09-12-0858"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/MBC.0b013e32802f7d91"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70005-7"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-10-386045"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1160\/TH12-08-0604"},{"key":"e_1_2_6_17_1","unstructured":"Tretten (coagulation factor XIII A\u2010subunit (recombinant)) [Internet]. 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