Gago Laura, Lourenço Maria Helena, Torres Rita P, Mourão Ana Filipa, Costa Maria Manuela, Branco Jaime C, Gonçalves Maria J
Rheumatology, Egas Moniz Hospital, Unidade Local de Saúde de Lisboa Ocidental, Lisbon, PRT.
Comprehensive Health Research Center (CHRC), Nova Medical School, Lisbon, PRT.
Cureus. 2024 Nov 6;16(11):e73149. doi: 10.7759/cureus.73149. eCollection 2024 Nov.
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is an uncommon but significant hematological disorder characterized by the presence of lupus anticoagulant (LA) and reduced levels of prothrombin (factor II). This syndrome presents a unique clinical paradox where patients may experience both a bleeding tendency due to hypoprothrombinemia and a prothrombotic state associated with LA. This syndrome should be suspected in the presence of increased coagulation times (prothrombin time and activated partial thromboplastin time) in association with the presence of LA. The dual risk between bleeding and thrombosis poses significant diagnostic and therapeutic challenges, particularly in deciding whether to use anticoagulation therapy, which could exacerbate bleeding, or withhold it, which could increase the risk of thrombosis. The authors report a case of a woman with LAHPS treated with rituximab. This case reinforces the importance of establishing guidelines for this disease.
狼疮抗凝物质-低凝血酶原血症综合征（LAHPS）是一种罕见但严重的血液系统疾病，其特征为存在狼疮抗凝物质（LA）且凝血酶原（因子II）水平降低。该综合征呈现出一种独特的临床矛盾现象，即患者可能因低凝血酶原血症而有出血倾向，同时又因LA而处于血栓形成前状态。当凝血时间（凝血酶原时间和活化部分凝血活酶时间）延长且伴有LA时，应怀疑该综合征。出血和血栓形成的双重风险带来了重大的诊断和治疗挑战，尤其是在决定是否使用抗凝治疗时，使用抗凝治疗可能会加重出血，而不使用则可能增加血栓形成的风险。作者报告了一例用利妥昔单抗治疗的LAHPS女性病例。该病例强化了为这种疾病制定指南的重要性。
