{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T21:23:05Z","timestamp":1777929785079,"version":"3.51.4"},"reference-count":42,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Thromb Hemost"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare, acquired coagulopathy syndrome. Here, we aim to summarize the clinical features of LAHPS to improve the understanding of the disease. The clinical data of 52 patients with LAHPS retrieved through PubMed from 2019 to 2023, supplemented with a local case of a child with LAHPS, were retrospectively analyzed, and the clinical characteristics were summarized. 56.6% of LAHPS patients were female, the median age at onset was 13.0 years (range, 1.2\u201385 years), and the median activity of factor II was 18.0% (range, 0.1\u201369%). 64.2% of LAHPS patients experienced hemorrhage, with 29.4% having multisite hemorrhage and 20.6% experiencing both nonsevere and severe hemorrhage. Most of the reported cases were secondary to autoimmune diseases (60.6%), followed by infections (33.3%). Corticosteroids were administered to 79.3% of patients with hemorrhage, and 90.6% of patients with LAHPS showed improvement. In conclusion, LAHPS is most commonly observed in female patients, particularly those under 18 years of age. LAHPS is characterized by hemorrhage, occurring at various sites and with varying degrees of severity, but the majority of patients improve with appropriate treatment and management.<\/jats:p>","DOI":"10.1055\/s-0044-1779739","type":"journal-article","created":{"date-parts":[[2024,2,23]],"date-time":"2024-02-23T23:57:11Z","timestamp":1708732631000},"page":"592-604","source":"Crossref","is-referenced-by-count":4,"title":["Lupus Anticoagulant-Hypoprothrombinemia Syndrome: Literature Review and Description of Local Case in a 3-Year-Old Chinese Girl"],"prefix":"10.1055","volume":"50","author":[{"given":"Bin","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ning","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chi","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,2,23]]},"reference":[{"issue":"01","key":"ref1","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s12959-021-00271-0","article-title":"Lupus anticoagulant hypoprothrombinemia syndrome associated with bilateral adrenal haemorrhage in a child: early diagnosis and intervention","volume":"19","author":"A Sakamoto","year":"2021","journal-title":"Thromb J"},{"issue":"08","key":"ref2","doi-asserted-by":"crossref","first-page":"3000605221115390","DOI":"10.1177\/03000605221115390","article-title":"Autoimmune hemolytic anemia 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Mulliez","year":"2015","journal-title":"Lupus"},{"issue":"08","key":"ref39","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1097\/MBC.0000000000000776","article-title":"Fatal pulmonary embolism and pulmonary hemorrhage in lupus anticoagulant hypoprothrombinemia syndrome: a case report and review of literature","volume":"29","author":"X Chen","year":"2018","journal-title":"Blood Coagul Fibrinolysis"},{"issue":"10","key":"ref40","doi-asserted-by":"crossref","first-page":"1933","DOI":"10.1007\/s00296-018-4127-9","article-title":"Lupus anticoagulant hypoprothrombinemia syndrome associated with systemic lupus erythematosus in children: report of two cases and systematic review of the literature","volume":"38","author":"R K Pilania","year":"2018","journal-title":"Rheumatol Int"},{"issue":"07","key":"ref41","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1093\/rheumatology\/kez453","article-title":"Interest of IgG and IgM antiprothrombin autoantibodies in the exploration 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