Background : Approximately 33% patients with severe hemophilia A (SHA) develops factor VIII (FVIII) inhibitor. Bleeding management in SHA with high titer FVIII inhibitor (> 5 BU) is challenging experience.
Case Presentation Summary : Objective: To describe case series of high titer FVIII inhibitor in SHA patients with massive bleeding. Cases: First case in 2018, a 15 years old boy with FVIII inhibitor titer was 11.86 BU suffered from ruptured pseudo-tumor of right knee. Patient underwent amputation of right knee, then was discharged with completely healed wound. Second case in 2017, a 13 years old boy with an unknown sourced of massive intraabdominal bleeding. His FVIII inhibitor titer was 518.4 BU. Patient underwent explorative laparotomy followed by colostomy. Immune tolerance induction (ITI) was conducted with extended half-life recombinant of FVIII. Patient did not survive due to hypovolemic shock caused by inadequate supply of bypassing agent. Third case in 2017, a 15 years old boy with pseudo-tumor of right illiopsoas muscle and compartment syndrome. His FVIII inhibitor titer was 40 BU. Patient underwent successful hematoma evacuation. Fourth case in 2015, a 9 years old boy with massive bleeding of the bladder due to oral cyclophosphamide consumption for 2 months. His FVIII inhibitor titer was 20.4 BU. Patient received uromithexan, underwent cystostomy followed by regular spooling to prevent blood clot. All patients received bypassing agent either rVIIa or activated prothrombin complex concentrate. They also received massive transfusion due to massive bleeding and antibiotic due to septicemia or local infection. The 1st and 2nd patients received cyclophosphamide as their immunosuppressant agent while others received methylprednisolone.
Learning Points/Discussion : Keys for managing bleeding in SHA patients with high titer inhibitor are adequate supply of bypassing agent and bleeding source control. Others important supportive therapy include massive transfusion, immunosuppressant agent and ITI to manage inhibitor.
Keywords: Severe Hemophilia A FVIII inhibitor Bypassing agent Massive bleeding
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