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Primary antiphospholipid syndrome: a report of two pediatric cases
F Falcini1, G Taccetti, S Trapani
1Department of Paediatrics, University of Florence, Italy.
Insights
Primary antiphospholipid syndrome can affect children, presenting with serious conditions like myocardial infarction and venous thrombosis. Testing for lupus anticoagulant and anticardiolipin antibodies is crucial in pediatric thromboembolic cases lacking clear autoimmune disease indicators.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Cardiology
Background:
- Primary antiphospholipid syndrome (APS) is a rare autoimmune disorder characterized by blood clots and pregnancy complications.
- While typically diagnosed in adults, pediatric cases of APS are increasingly recognized, presenting unique diagnostic challenges.
Observation:
- Two pediatric cases of primary APS are presented: a 6-year-old girl with myocardial infarction, chorea, livedo reticularis, and thrombocytopenia.
- A 7-year-old boy experienced recurrent intracranial hypertension and deep venous thrombosis, with high-titer IgG anticardiolipin antibodies (aCI) detected.
Findings:
- The cases highlight that primary APS can manifest with severe thrombotic events in children.
- Circulating lupus anticoagulant (LAC) and IgG anticardiolipin antibodies (aCI) were key indicators in both pediatric patients.
Implications:
- These findings underscore the importance of considering primary APS in children presenting with unexplained thromboembolic phenomena.
- Routine screening for LAC and aCI antibodies is recommended in pediatric patients with thrombotic events, even when other autoimmune disease criteria are absent.
Abstract:
Two cases of primary antiphospholipid syndrome are described. A girl presented with myocardial infarction at the age of 6. afterward developed chorea, livedo reticularis, thrombocytopenia and circulating lupus anticoagulant (LAC). A boy, age 7, had an episode of intracranial hypertension and a deep venous thrombosis of a lower left limb, both recurrent in the following years. A high titer of IgG anticardiolipin antibodies (aCI) was detected. These observations suggest that both LAC and aCI tests should be performed in children with thromboembolic phenomena when the criteria for a definite autoimmune disease are lacking.