RECURENT THROMBOSIS IN AN ADOLESCENT WITH ANTIPHOSPHOLIPID SYNDROME AND MULT IPLE RISK FACTORS

Inherited thrombophilia increases the risk of a first thrombotic event and venous thromboembolism. Coexistence of inherited thrombophilia and autoimmune disorders (antiphospholipid syndrome) can lead to severe recurrent venous thrombosis. A sixteen-year-old boy with a family history of pulmonary embolism developed multiple venous thromboses (deep vein thrombosis) and severe post-thrombotic syndrome. The first thrombotic event occurred after trauma. The patient was considered carries for inherited thrombophilia. Subsequent investigations revealed deficiency of protein C, S and persistence of lupus anticoagulant. High level of homocysteine was also determined. Each episode of acute respiratory viral infection resulted in thrombosis. Postthrombotic syndrome occurred due to the failure of recanalization. Failure of recanalization is now recognized as an independent predictor of recurrent deep vein thrombosis. Doppler ultrasound detected jugular vein thrombosis and left and right femoral vein thrombosis. Anticoagulants were prescribed, but they did not prevent the thrombosis. The patient took warfarin (INR= 2.5-3.0, sometimes 4.0), but it was not effective. Thus, the patient was considered to have inherited thrombophilia and antiphospholipid syndrome disorder combination. Low molecular weight heparin was prescribed for the prevention of thrombosis (Anti-X-a=0.6-1.0), and hydroxychloroquine was prescribed as a mild immunosuppressive drug. Venous thromboembolism is a rare disease in children. Commonly, it is a multifactorial condition caused by both genetic and acquired risk factors. Patients with family history and earlyonset thrombosis need screening for inherited thrombophilia, autoimmune disorders or oncology. In addition, the impact of risk factors must be taken into consideration. The length.f treatment and preventive therapy remains unclear and depends on the risk factors.