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KMID : 0387820140210020145
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Clinical Pediatric Hematology-Oncology
2014 Volume.21 No. 2 p.145 ~ p.148
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Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report
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Sul Yeon-Ah
Lee Joo-Hoon
Park Young-Seo
Koh Kyung-Nam
Im Ho-Joon
Seo Jong-Jin
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Abstract
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The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often pre-sented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell’s viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anti-coagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with im-munosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.
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KEYWORD
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Lupus anticoagulant, Hypoprothrombinemia, Systemic lupus erythematosus
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