Hypoprothrombinemia-lupus anticoagulant syndrome: a case report and literature review
10.3760/cma.j.cn121090-20241129-00496
- VernacularTitle:低凝血酶原血症-狼疮抗凝物综合征1例并文献复习
- Author:
Panjing WANG
1
;
Wei LIU
;
Ting SUN
;
Yuhua WANG
;
Renchi YANG
;
Lei ZHANG
;
Feng XUE
Author Information
1. 中国医学科学院血液病医院（中国医学科学院血液学研究所），血液与健康全国重点实验室，国家血液系统疾病临床医学研究中心，细胞生态海河实验室，天津市血液病基因治疗研究重点实验室，中国医学科学院血液病基因治疗重点实验室，天津 300020
- Publication Type:Journal Article
- From:
Chinese Journal of Hematology
2024;45(S1):89-91
- CountryChina
- Language:Chinese
-
Abstract:
The Hypoprothrombinemia-Lupus Anticoagulant Syndrome (HLAS) is a rare coagulation disorder, typically presenting with bleeding manifestations. It is characterized by decreased prothrombin activity and the presence of lupus anticoagulant, but laboratory findings are complex, which can delay diagnosis and treatment. This report describes the diagnosis and management of a HLAS patient who was an 11-year-old girl with recurrent bleeding. Coagulation tests revealed prolonged APTT and PT, decreased prothrombin activity, and immediate inhibitory antibodies in the APTT correction test. Lupus anticoagulant, antiphospholipid antibodies, anti-β 2-glycoprotein 1 antibodies, and phosphatidylserine/prothrombin (aPS/PT) antibodies were positive, leading to a diagnosis of Hypoprothrombinemia-Lupus Anticoagulant Syndrome. The patient was also diagnosed with systemic lupus erythematosus. Following corticosteroid therapy, prothrombin activity returned to normal.
