Lupus anticoagulant-hypoprothrombinemia syndrome with a false-positive test for coagulation factor inhibitors

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  • MORINAGA Shingo
    Department of Pediatrics, National Hospital Organization Kumamoto Medical Center.
  • IDE Yoko
    Department of Pediatrics, National Hospital Organization Kumamoto Medical Center.
  • KUSUMOTO Masaru
    Department of Pediatrics, National Hospital Organization Kumamoto Medical Center.
  • OGATA Mika
    Department of Pediatrics, National Hospital Organization Kumamoto Medical Center.
  • TAKAKI Kazutaka
    Department of Pediatrics, National Hospital Organization Kumamoto Medical Center.
  • MATSUMOTO Emiko
    Department of Clinical Laboratory for Blood Examination, National Hospital Organization Kumamoto Medical Center

Bibliographic Information

Other Title
  • 複数の凝固因子インヒビター偽陽性を示したループスアンチコアグラント・低プロトロンビン血症症候群
  • 症例報告 複数の凝固因子インヒビター偽陽性を示したループスアンチコアグラント・低プロトロンビン血症症候群
  • ショウレイ ホウコク フクスウ ノ ギョウコ インシ インヒビター ギ ヨウセイ オ シメシタ ループスアンチコアグラント ・ テイプロトロンビン ケツショウ ショウコウグン

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Abstract

We report a case of a 1-year-old boy diagnosed with lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS), which is a rare disorder. His initial presentation of sinusitis was accompanied by hemorrhagic episodes including ecchymoses and epistaxis 6 months after antibiotic therapy. Laboratory results revealed prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT) that did not correct with mixing studies. Factors II, VIII, IX, X, XI, and XII activities were 20%, 44%, 42.5%, 59%, 4%, and 10%, respectively. The Bethesda inhibitor assay showed inhibitors against multiple coagulation factor. APTT, mixing studies, diluted Russell's viper venom time, and the Bethesda inhibitor assay detected LA. LA-HPS with a suspected false-positive test for coagulation factor inhibitors was diagnosed. Bleeding stopped and results of coagulation studies returned to normal without therapy 2 months after onset of the disease.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 53 (7), 716-720, 2012

    The Japanese Society of Hematology

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