Lupus Anticoagulant-Hypoprothrombinemia Syndrome: A Duality Between Thrombosis and Hemorrhage

Abstract

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.

Keywords: blood coagulation factors; coagulation defect; lupus anticoagulant-hypoprothrombinemia syndrome; secondary antiphospholipid syndrome; systemic lupus erythematosus.