Question

A boy with hemophilia A with inhibitors comes to your center after a road traffic accident and requires urgent surgery. How will you manage surgery in this patient?

Hint:

Activated recombinant factor VII (rFVII and bypassing agents are critical in managing surgery for patients with hemophilia A and inhibitors. Careful monitoring is required to prevent both bleeding and thrombosis.

Answer 1

Step 1: Preoperative Management.
In a patient with hemophilia A and inhibitors, managing surgery requires careful planning due to the risk of bleeding. The presence of inhibitors makes it more challenging to control bleeding, as factor VIII therapy may not be effective. The following steps are essential: 1. Activated recombinant factor VII (rFVII: The primary treatment for bleeding episodes in patients with inhibitors is activated recombinant factor VII (rFVII. This can be used to control bleeding during surgery.
2. Bypassing agents: Another option is to use bypassing agents like activated prothrombin complex concentrate (aPCC), which can help reduce bleeding by bypassing the blocked factor VIII activity.
3. Desmopressin (DDAVP): If the patient has a mild form of hemophilia A, desmopressin (DDAVP) may be considered to stimulate the release of endogenous factor VIII, though this is less effective in patients with inhibitors.

Step 2: Intraoperative Monitoring.
During surgery, close monitoring of the patient’s coagulation status is essential. This may include regular measurements of activated partial thromboplastin time (aPTT), and Factor VII levels to assess hemostasis. The goal is to maintain adequate clotting while avoiding over-correction that could lead to thrombosis.