Abstract Number: PB0790
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Acquired Hemorrhagic Coagulation Disorders
Background: Acquired factor VIII (FVIII) deficiency is a rare and potentially fatal coagulopathy with limited experience in patients undergoing major surgical procedures. Bypassing agents and recombinant porcine FVIII (rpFVIII) are hemostatic drugs used to treat and prevent bleeding in patients with acquired FVIII deficiency.
Aims: Describe 3 clinical cases of patients diagnosed with acquired FVIII deficiency that underwent a major surgical procedure managed with rpFVIII.
Methods: Retrospective record review of 3 acquired FVIII patients at the Gulf States Hemophilia and Thrombophilia Center of Houston, Texas who underwent a major surgical procedure with active inhibitor.
Results: Only one dose of rpFVIII was given preoperatively for each patient. Two patients (#1 and #2) demonstrated an excellent response to rpFVIII with an average estimated blood loss of 950 cc. Poor response experience by Patient #3 was most likely related to antibodies against rpFVIII as the peak FVIII level at 1 hour was only 4.6%. However, this patient experience no excessive bleeding, most likely due to the effect of emicizumab. No thromboembolic complications were reported.
Conclusions: Recombinant porcine FVIII is a safe and effective hemostatic drug for patients with acquired factor VIII deficiency undergoing major surgery. Close monitoring of FVIII levels is important to assess efficacy and to adjust dosing. In our experience lower dosing of rpFVIII than recommended by manufacture are needed for major surgical procedures. Pharmacokinetic study with rpFVIII is recommended.
| Gender/Age | Secondary DX | Peak Inhibitor | Inhibitor at time of surgery | Immunosuppressive Treatment | Procedure | Initial dose of rpFVIII | Subsequent rpFVIII doses | Estimated Blood Loss | |
| Patient #1 | F/24 | Pregnancy | 79 B.U. | 2.70 B.U. | Prednisone | Cesarean Section | 100 mg/kg | – | 600 cc |
| Patient #2 | F/53 | RA | 17.6 B.U. | 5.60 B.U. | Prednisone | Cardiac (valve replacement) | 100 mg/kg | 50 IU/kg q 6 hrs (x5 doses) | 1300 cc |
| Patient #3 | M/90 | Idiopathic AFib, CAD, CHF | 380 B.U. | 55 B.U. | Rituximab Mycophenolic acid *Emicizumab | Knee Arthrotomy | 100 mg/kg | – | 100 cc |
[Table 1.]
To cite this abstract in AMA style:
Escobar M, Montanez N, Aboshady I, Chong K. Experience with Recombinant Porcine FVIII in Patients with Acquired Factor VIII Deficiency Undergoing Major Surgery [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/experience-with-recombinant-porcine-fviii-in-patients-with-acquired-factor-viii-deficiency-undergoing-major-surgery/. Accessed September 24, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/experience-with-recombinant-porcine-fviii-in-patients-with-acquired-factor-viii-deficiency-undergoing-major-surgery/