PB1268 - Management Patient Severe Hemophilia A with Pseudotumor at North Borneo Public Hospital : A Case Report

Background: Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild trauma. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor.

Aims: The risk of uncontrollable hemorrhage during surgery is preventable with the availability of Factor VIII, management should be carried out by a multidisciplinary surgical team, including hematologists, orthopedic surgeons, and rehabilitation physicians.

Methods: A 35-year-old, 50kg, male suffering from Hemophilia A. had concultation because recurrent swelling and hematoma in the left distal femur and left knee for 4 years prior to admission and unable to walk due to chronic sinovitis. The lump increases in dimension (2cm to 12cm). Laboratory findings revealed a APTT 102 seconds, and Factor VIII < 1%. The radiologic study showed expansile and lytic lesion democrated by calcification from left fermoralis distal to knee with cortical thinning and soft tissue swelling around it. The diagnosed is hemophilic pseudotumor of the left femur and knee. It was decided to evacuate the hematoma. The initial dose of the factor VIII concentrates was 1500 units once daily. On day 2 and 3, factor VIII concentrates was given 1500 units twice daily, followed by 1000 units twice daily on day 4 to day 7. In patients, no visible improvement but we cannot perform inhibitor test because limited cost bye government insurance. Then, added bypassing agents aPCC 2500 units/12 hours for 1 week, Pain Management using Paracetamol and supportive treatment PRICE. After 1 week the bleeding was managed well

Results: Pseudotumors hemophilia may occur in different degrees of hemophilia, especially those with severe hemophilia. The risk of uncontrollable hemorrhage during surgery is preventable with the availability of Factor VIII or bypassing agents to aid in clotting

Conclusion(s): To conclude, we successfully managed a severe hemophilia A with pseudotumor. Use management hemophilia A guidelines