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.