Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

  • Kim, Ji Soo (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Kim, Min Jae (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Bae, E. Young (Department of Pediatrics, The Catholic University of Korea College of Medicine) ;
  • Jeong, Dae Chul (Department of Pediatrics, The Catholic University of Korea College of Medicine)
  • 투고 : 2013.02.13
  • 심사 : 2013.09.13
  • 발행 : 2014.04.15
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초록

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

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참고문헌

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  3. Lupus anticoagulant hypoprothrombinemia syndrome associated with severe thrombocytopenia in a child vol.64, pp.6, 2014, https://doi.org/10.1002/pbc.26357
  4. Lupus anticoagulant hypoprothrombinemia syndrome associated with systemic lupus erythematosus in children: report of two cases and systematic review of the literature vol.38, pp.10, 2018, https://doi.org/10.1007/s00296-018-4127-9
  5. Rituximab use in pediatric lupus anticoagulant hypoprothrombinemia syndrome - report of three cases and review of the literature vol.27, pp.7, 2014, https://doi.org/10.1177/0961203317751853
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  7. An Extremely Rare Cause of Prolonged Menstruation: Lupus Anticoagulant-Hypoprothrombinemia Syndrome vol.40, pp.6, 2014, https://doi.org/10.1080/01443615.2019.1637406
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