ABSTRACT
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.
Subject(s)
Child , Child, Preschool , Humans , Antiphospholipid Syndrome , DNA , Lupus Coagulation Inhibitor , Lupus Erythematosus, Systemic , Mass Screening , Partial Thromboplastin Time , Plasma , Prothrombin , Prothrombin Time , Risk FactorsABSTRACT
Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.
Subject(s)
Child , Child, Preschool , Humans , Antiphospholipid Syndrome , DNA , Lupus Coagulation Inhibitor , Lupus Erythematosus, Systemic , Mass Screening , Partial Thromboplastin Time , Plasma , Prothrombin , Prothrombin Time , Risk FactorsABSTRACT
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.