Yoshika Masamichi, Komiyama Yutaka, Yoshida Mika, Naito Sumiyoshi, Ieko Masahiro, Tsuta Koji
Rinsho Byori. 2016 Jun;64(6):631-635.
Lupus anticoagulant-hypothrombinemia syndrome (LAHS) is a rare disease involving hemorrhagic diathe- sis due to hypothrombinemia with lupus anticoagulant. We report a 28-week-pregnant woman at twenty years of age, who had been hospitalized with jaundice. In laboratory data, AST, ALT, and bilirubin were elevated and the prothrombin time (PT) and activated partial thromboplastin time (APTT) were prolonged. Although the liver failure was improved after she delivered a baby by Caesarean section, postoperative intraperitoneal bleeding persisted. The diagnosis by liver biopsy was autoimmune hepatitis. Although the bleeding was stopped on the seventh postoperative day, the prolongation of PT and APTT remained. LA was positive in the diluted Russell's viper venom time. Anti-cardiolipin and anti-beta-2-glycoprotein anti- bodies were also positive. The prothrombin activity was reduced. A high titer of phosphatidylserine- dependent antiprothrombin antibody (aPS/PT), which causes bleeding, was observed. Based on these data, she was diagnosed with LAHS. The liver dysfunction and prolongation of PT and APTT were normalized following the administration of corticosteroids. In this case, aPS/PT may have contributed to the pathological physiology of LAHS. [Case Report].
狼疮抗凝物-低凝血酶血症综合征（LAHS）是一种罕见疾病，因狼疮抗凝物导致的低凝血酶血症而引发出血素质。我们报告一例20岁孕28周的孕妇，因黄疸入院。实验室检查数据显示，谷草转氨酶（AST）、谷丙转氨酶（ALT）及胆红素升高，凝血酶原时间（PT）和活化部分凝血活酶时间（APTT）延长。尽管她剖宫产分娩后肝功能衰竭有所改善，但术后腹腔内出血持续存在。肝活检诊断为自身免疫性肝炎。尽管术后第7天出血停止，但PT和APTT仍延长。稀释蝰蛇毒时间检测狼疮抗凝物（LA）呈阳性。抗心磷脂抗体和抗β2糖蛋白抗体也呈阳性。凝血酶原活性降低。观察到高滴度的依赖磷脂酰丝氨酸的抗凝血酶原抗体（aPS/PT），该抗体可导致出血。基于这些数据，她被诊断为LAHS。给予糖皮质激素后，肝功能障碍及PT和APTT延长恢复正常。在此病例中，aPS/PT可能参与了LAHS的病理生理过程。[病例报告]
