Pediatric Rheumatology Online Journal → July 2003 → Anti-Phospholipid Antibody Syndrome→ Abstract #117
ANTIPHOSPHOLIPID (APL) ANTIBODIES AND THROMBOSES IN A PRETERM BOY
E. B. Nordal, M. N. Fredriksen, C. A. Klingenberg
Antiphospholipid syndrome is a rare, but important cause of thrombotic disease in the newborn. We report of a preterm boy of 34 weeks. During the first week of life he developed intermittent paleness of the left leg, thrombocytopenia, and repeated episodes of melena. Further he developed an ileus, which led to resection of a third of the ileum. Doppler ultrasound showed reduced left femoral artery flow and magnetic resonance imaging (MRI) angiography demonstrated a narrowing of the femoral artery, compatible with an arterial thrombosis. Ultrasound and MRI angiography also revealed a two-centimeter long thrombus in the inferior vena cava. Echocardiography, Doppler sonography of the carotids and MRI of cerebrum were all normal. Treatment with low weight molecular heparin was started. He underwent a stoma closure after 8 weeks. After eight months ultrasound showed calcified remnants of the venous thrombus. On follow-up one year later he is a healthy child without medication.
Microscopy of the resected bowel revealed necrosis, inflammation and microthromboses. Laboratory parameters for thrombophilia, vasculitis and SLE were all normal. However, in the mother we detected a high level of cardiolipin IgG antibodies 75 GPL U/ml. The child initially had cardiolipin IgG 1 GPL U/ml, but on follow-up cardiolipin antibodies were completely negative.
We believe that his venous and arterial thromboses were caused by maternal IgG class antibodies transferred over placenta. Cerebral thromboses in newborns with increased level of APL antibodies are previously reported. APL syndrome is characterized by high levels of APL antibodies and thrombotic events or other clinical manifestations. The prognosis is usually good as maternal antibodies in newborns degrade spontaneously.