Severe deep vein thrombosis and pulmonary embolism in a paediatric patient with primary antiphospholipid syndrome

Abstract

Antiphospholipid syndrome (APS) is defined by the persistent presence of moderate to high serum levels of antiphospholipid antibodies (aPL) in association with recurrent thrombotic events, pregnancy loss or both. APS may occur as a primary disorder or secondary to an underlying autoimmune disease. Half of all paediatric cases are of the secondary type, and approximately 80% of these have systemic lupus erythematosus as the underlying disease. Acute anticoagulant therapy is recommended for first episode of deep vein thrombosis, and thrombolytic therapy is recommended only for life- or limb-threatening thrombosis. We experienced a 13-year-old girl who was diagnosed as having primary APS with triple positivity for aPL, i.e. plasma lupus anticoagulant, anticardiolipin antibodies and anti-β2 glycoprotein I antibodies. She did not respond to initial treatment with anticoagulant therapy including unfractionated heparin and warfarin, and dyspnoea and chest pain were getting worse. Contrast computed tomography on day 12 revealed exacerbation of thromboembolism (TE). Owing to deterioration in patient condition, urokinase was added immediately after the placement of an inferior vena cava filter. After initiating thrombolytic therapy, her oxygenation and symptoms showed gradual improvement. In conclusion, it is obvious that refractory APS patients with triple-positive are at a high risk of recurrent thrombotic event and a first thrombotic event; the treatment of TE in the acute phase should be guided by careful risk-assessment based on the patient’s aPL profile.