Stopping the bleed: A hemophilia B success story

Hemophilia B is a rare X-linked recessive coagulation disorder caused by a deficiency in factor IX (FIX) activity. Intracranial hemorrhage (ICH) is a life-threatening complication of hemophilia. Approximately 20,000 cases are reported in India.[1] The severity of hemophilia B is classified based on FIX activity levels: mild (5%–40%), moderate (1%–5%), and severe (<1%). Treatment involves replacing the deficient FIX with recombinant or plasma-derived concentrates.[2] A 3-year-old male, weighing 10 kg, with hemophilia B presented with seizures, lethargy, and a Glasgow Coma Scale of 8 (E2V2M4). Radiological imaging revealed a subdural hematoma with a midline shift. The patient was scheduled for emergency decompressive craniotomy (ASA III E). Baseline FIX levels were 4%, and hemoglobin was 7 g/dL (normal FIX levels in nonhemophilic patients: 50%–150%). Preoperatively, the patient received 1000 IU (100 IU/kg) of recombinant FIX (Alprolix™) through slow intravenous infusion, achieving a posttransfusion FIX level of 102.8% to minimize bleeding risk. The surgery was uneventful, with 150 mL of blood loss and a urine output of 15 mL. Intraoperatively, the patient received 150 mL of packed red blood cells. Postoperative medications included intravenous tranexamic acid (15 mg/kg), levetiracetam (10 mg/kg), dexamethasone (1 mg), and paracetamol (15 mg/kg). The patient was transferred to the intensive care unit without extubation. On postoperative day (POD) 1, the child was extubated without neurological deficits. He was discharged on POD 4 with a plan for hematological follow-up. Prophylactic FIX administration is critical to prevent perioperative bleeding. Guidelines recommend FIX dosing of 100–120 IU/kg for severe bleeding and 50–60 IU/kg for hemarthrosis. In cranial trauma, doses of 50–100 IU/kg are advised. Pre- and postoperative FIX levels must be maintained in consultation with a hematologist. Perioperative precautions include avoiding regional anesthesia, intramuscular injections, aspirin, and nonsteroidal anti-inflammatory drugs. Fresh-frozen plasma or cryo-poor plasma contains approximately 1 IU/mL of FIX, while FIX concentrates are dosed based on the principle that 1 unit/kg increases plasma FIX by approximately 0.7%–1%. For example, administering 50 units/kg raises FIX levels by 35%–50%.[3] Managing pediatric patients with rare coagulation disorders like hemophilia B, especially in the context of ICH, requires a multidisciplinary approach involving hematologists, anesthesiologists, and neurosurgeons. FIX replacement therapy, meticulous surgical technique, and specialized perioperative care are essential to mitigate bleeding risks and optimize outcomes. In this case, although intraoperative bleeding was manageable, the potential for significant hemorrhage underscores the need for comprehensive and coordinated care. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.