Hypoprothrombinemia

Hypoprothrombinemia-lupus anticoagulant syndrome (HLAS) is a rare disease in which patients present with varying degrees of bleeding and positive lupus anticoagulant with reduced prothrombin on laboratory tests. This article reports a case of HLAS in a middle-aged woman with recurrent gingival bleeding and epistaxis as the first presentation. After admission, tests revealed prolonged prothrombin time (PT), activated partial thromboplastin time (APTT), and reduced coagulation factor II activity, and positive lupus anticoagulant (LA). Meanwhile, the patient had symptoms of dry mouth and dry eyes for a long time, and the examination of autoantibodies, tear secretion test and salivary gland emission computed tomography (ECT) were consistent with the diagnosis of Sjogren's syndrome. The final diagnosis was HLAS secondary to Sjogren's syndrome. After treatment with methylprednisolone and cyclophosphamide, the coagulation disorder gradually improved, and no recurrent bleeding occurred. HLAS is a rare clinical case, which reminds medical staff to be alert to the possibility of HLAS when encountering patients with unexplained prolonged APTT and PT and positive lupus anticoagulant. 低凝血酶原血症-狼疮抗凝物综合征(hypoprothrombinemia-lupus anticoagulant syndrome，HLAS)是临床罕见的疾病，患者常表现为不同程度的出血，实验室检查可发现狼疮抗凝物阳性伴凝血酶原降低。本文报道1例中年女性HLAS病例，患者以反复牙龈出血、鼻衄为首发表现，入院后检查发现凝血酶原时间(prothrombin time，PT)和活化部分凝血活酶时间(activated partial thromboplastin time，APTT)延长，凝血因子II活性降低，狼疮抗凝物(lupus anticoagulant，LA)阳性。同时，患者存在长时间口干、眼干症状，经自身抗体、泪液分泌试验及唾液腺发射计算机断层显像(emission computed tomography，ECT)等检查符合干燥综合征的诊断。最终诊断为干燥综合征继发的HLAS。经过甲泼尼龙及环磷酰胺治疗后凝血象逐渐改善，未再出血。HLAS为临床罕见病，医务人员在遇到不明原因APTT和PT延长且LA阳性患者，需警惕HLAS的可能。.