Asia Pacific Journal of Pediatric and Child Health

Abstract Ref Number = APCP934

Poster Presentation

MANAGEMENT OF HIGH TITER FACTOR VIII INHIBITOR IN SEVERE HEMOPHILIA A WITH MASSIVE BLEEDING A CASE SERIES

Melati Arum Satiti,Fitri Primacakti,Novie Amelia Chozie,Djajadiman Gatot RSUPN Cipto Mangunkusumo

Background : Introduction: Around 33% patients with severe hemophilia A (SHA) develops factor VIII (FVIII) inhibitor. Management of bleeding in SHA can be a challenging experience especially with high titer FVIII inhibitor, > 5 Bethesda Unit (BU). Objective: To describe case series of high titer FVIII inhibitor in SHA with massive bleeding. Case Presentation Summary : Cases: First case in 2018, a 15 years old boy with SHA and ruptured pseudotumor of right knee. Blood examination revealed FVIII inhibitor of 11.86 BU. Patient received intravenous cyclophosphamide 100 mg/m2 once a week, massive transfusion, recombinant of FVIIa (rFVIIa), activated prothrombin complex concentrate, and amputation of right knee. Patient was discharged with completely healed wound. Second case in 2017, a 13 years old boy with SHA and an unknown sourced of massive intraabdominal bleeding. Blood examination revealed FVIII inhibitor of 518.4 BU. Patient received intravenous cyclophosphamide 100 mg/m2 once a week, massive transfusion, rFVIIa, and underwent explorative laparotomy followed by colostomy. He also underwent immune tolerance induction (ITI) 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 2015, a 9 years old boy with SHA and massive bleeding of the bladder due to oral cyclophosphamide consumption for 2 months. Blood examination revealed FVIII inhibitor of 20.4 BU. Patient received methylprednisolone, uromithexan, cystostomy followed by regular spooling to prevent blood clot, massive transfusion, and rFVIIa. Patient eventually recovered and continued his outpatient follow–up at different hospital. All patients suffered from septicamia and received antibiotic. Learning Points/Discussion : Conclusion: Keys for manage bleeding in SHA patients with high titer inhibitor are adequate supply of bypassing agent and stopped the bleeding source. Others important supportive therapy include immunosuppressant agent and ITI to manage inhibitor and massive transfusion.

Keywords: Severe Hemophilia A FVIII inhibitor Bypassing agent Massive bleeding