Korean J Pediatr.  2014 Apr;57(4):202-205. 10.3345/kjp.2014.57.4.202.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome

• Kim JS
• Kim MJ
• Bae EY
• Jeong DC

• KMID: 2284686
• DOI: http://doi.org/10.3345/kjp.2014.57.4.202

Abstract

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.

Keyword

Lupus anticoagulant-hypoprothrombinemia syndrome; Steroids; Immunoglobulins

MeSH Terms

Adolescent
Antibodies
Antibodies, Antinuclear
Blood Transfusion
Female
Hemorrhage*
Humans
Hypoprothrombinemias*
Immunoglobulins
Immunosuppressive Agents
Lupus Coagulation Inhibitor*
Methylprednisolone
Pancytopenia
Partial Thromboplastin Time
Plasma
Prothrombin
Prothrombin Time
Rare Diseases
Steroids
Vitamin K
Antibodies
Antibodies, Antinuclear
Immunoglobulins
Immunosuppressive Agents
Lupus Coagulation Inhibitor
Methylprednisolone
Prothrombin
Steroids
Vitamin K