Lupus anticoagulant hypoprothrombinemia syndrome associated with a hemorrhagic ovarian cyst in a girl with systemic lupus erythematosus: a case report
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare entity characterized by the presence of lupus anticoagulant (LA) and prothrombin (factor II) deficiency. It may cause severe bleeding contrary to classical antiphospholipid syndrome. Here, we report a case of LAHPS presenting with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus (SLE) nephritis. She had been followed up for 8 years. Her first manifestation of SLE was prolonged gingival bleeding after tooth extraction at 9 years of age. During the follow-up period, she had neither severe bleeding nor thrombotic complications despite a positive LA and a prolonged activated partial thromboplastin time (aPTT). At this visit, the patient presented with colicky abdominal pain, a hemorrhagic ovarian cyst, a prolonged prothrombin time, a prolonged aPTT, a low factor II level, and a positive LA, leading to the diagnosis of LAHPS. While a hemorrhagic ovarian cyst resolved completely in 3 months, she received oral pill, transfusions of red blood cells and plasma, and intravenous cyclophosphamide pulse therapy in combination with glucocorticoids due to persistent menorrhagia, anemia, prolonged aPTT, and lupus flaring. Thus, LAHPS needs to be considered in SLE patients with positive LA and prolonged aPTT.
Abstract
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) is a rare entity characterized by the presence of lupus anticoagulant (LA) and prothrombin (factor II) deficiency. It may cause severe bleeding contrary to classical antiphospholipid syndrome. Here, we report a case of LAHPS presenting with a hemorrhagic ovarian cyst in a 17-year-old girl with systemic lupus erythematosus (SLE) nephritis. She had been followed up for 8 years. Her first manifestation of SLE was prolonged gingival bleeding after tooth extraction at 9 years of age. During the follow-up period, she had neither severe bleeding nor thrombotic complications despite a positive LA and a prolonged activated partial thromboplastin time (aPTT). At this visit, the patient presented with colicky abdominal pain, a hemorrhagic ovarian cyst, a prolonged prothrombin time, a prolonged aPTT, a low factor II level, and a positive LA, leading to the diagnosis of LAHPS. While a hemorrhagic ovarian cyst resolved completely in 3 months, she received oral pill, transfusions of red blood cells and plasma, and intravenous cyclophosphamide pulse therapy in combination with glucocorticoids due to persistent menorrhagia, anemia, prolonged aPTT, and lupus flaring. Thus, LAHPS needs to be considered in SLE patients with positive LA and prolonged aPTT.
참고문헌 (14)
- Chung CH, "Lupus anticoagulant-hypoprothrombinemia in healthy adult", Korean J Intern Med, 23, pp.149~151 (2008)
- Mulliez SM, "Lupus anticoagulant-hypoprothrombinemia syndrome : report of two cases and review of the literature", Lupus, 24, pp.736~745 (2015)
- Atsumi T, "Clinical relevance of antiprothrombin antibodies", Autoimmun Rev, 1, pp.49~53 (2002)
- Montes de Oca MA, "Thrombosis in systemic lupus erythematosus : a French collaborative study", Arch Dis Child, 66, pp.713~717 (1991)
- Rapaport SI, "A plasma coagulation defect in systemic lupus erythematosus arising from hypoprothrombinemia combined with antiprothrombinase activity", Blood, 15, pp.212~227 (1960)
- Hoffman M, "Rethinking the coagulation cascade", Jpn J Thromb Hemost, 16, pp.70~81 (2005)
- Lackner KJ, "Pathogenesis of the antiphospholipid syndrome revisited : time to challenge the dogma", J Thromb Haemost, 14, pp.1117~1120 (2016)
- Mazodier K, "Lupus anticoagulant-hypoprothrombinemia syndrome : report of 8 cases and review of the literature", Medicine(Baltimore), 91, pp.251~260 (2012)
- Kocheril AP, "Pediatric systemic lupus erythematosus with lupus anticoagulant hypoprothrombinemia syndrome : a case series with review of literature", Lupus, 30, pp.641~648 (2021)
- Bajaj SP, "A mechanism for the hypoprothrombinemia of the acquired hypoprothrombinemia-lupus anticoagulant syndrome", Blood, 61, pp.684~692 (1983)
- "Lupus anticoagulants" (2024)[URL]
- Tarr T, "Clinical thrombotic manifestations in SLE patients with and without antiphospholipid antibodies : a 5-year follow-up", Clin Rev Allergy Immunol, 32, pp.131~137 (2007)
- Cetin Gedik K, "Rituximab use in pediatric lupus anticoagulant hypoprothrombinemia syndrome : report of three cases and review of the literature", Lupus, 27, pp.1190~1197 (2018)
- Lim W, "Antiphospholipid syndrome", Hematology Am Soc Hematol Educ Program, 2013, pp.675~680 (2013)