Manager of pediatric hematology department Manager of hemophilia center Samsun, Samsun, Turkey
Background: Antiphospholipid syndrome(APS) is a rare autoimmune disease of unknown etiology that represents the leading cause of acquired thromboembolism and recurrent miscarriage. It is characterized by the persistently elevated presence of pathogenic antiphospholipid autoantibodies against cardiolipin, ß2-glycoprotein-I and/or a positive lupus anticoagulant test. Although it is more common in adults, APS can sometimes occur in the neonatal period and childhood. Adult-onset classification criteria have poor validity for the pediatric population (where pregnancy-related complications are rare) and, as a result, the prevalence of the disease in childhood is difficult to assess. Thromboembolic events in children include stroke and pulmonary embolism, as well as deep vein thrombosis, which can cause serious long-term disability.
Aims: Our aim in this study is to retrospectively evaluate pediatric APS cases diagnosed in our clinic.
Methods: Eight pediatric patients were diagnosed with APS in our clinic. Six of the patients were girls and two were boys. Ages at diagnosis were between 10 and 16.
Results: All patients were diagnosed with APS secondary to SLE. Presentation findings were skin rash, thrombocytopenia, involuntary movements(chorea) or thromboembolic events. All patients were diagnosed in the pediatric hematology department and were followed up in the rheumatology department due to the underlying diagnosis of SLE. Follow-up periods vary between 1-20 years. All patients are receiving anticoagulant treatment. Thromboembolic events have been observed when treatment compliance is disrupted.
Conclusion(s): Pediatric APS cases present with hematological findings and are diagnosed by pediatric hematologists. Diagnosis of pediatric APS is more difficult because diagnostic criteria were developed for adults. Since pregnancy morbidity does not usually occur in children, it is difficult to complete the diagnostic criteria. Diagnostic criteria for pediatric APS cases should be redefined. Therefore, we believe that it would be beneficial to collect and evaluate cases retrospectively and prospectively in a multicenter manner for this rare disease.
