P026: ACQUIRED FACTOR VIII DEFICIENCY AND THE VASCULAR PATIENT
LaTasha D Moore, MD; University of Florida-Gainesville
INTRODUCTION: Classical hemophilia is an inherited form of coagulopathy associated with excessive bleeding beginning at an early age. In contrast, acquired hemophilia is a rare condition caused by the production of autoantibodies that inactivate Factor VIII presenting in adulthood. Diagnosis is based on having low plasma levels of Factor VIII and the presence of a time-dependent inhibitor. Major vascular cases are commonly associated with massive blood loss. Management of major intraoperative bleeding can pose a challenge as these patients do not respond to Factor VIII replacement.
CASE REPORT: A 66-year-old, 41-kg female with a history of acquired hemophilia A, calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome, coronary artery disease, and hypertension presented to our institution for a femoro-femoral bypass. She was diagnosed with acquired hemophilia A at the age of 64 after experiencing recurrent episodes of epistaxis. Her prior medication list included Prednisone, Cellcept, and Rituxan for immunosuppression; all were ineffective at reducing her inhibitor concentration. Prior to her scheduled surgery, hematology was consulted for perioperative recommendations and Obizur (recombinant porcine factor VIII) was prescribed to increase Factor VIII levels. Obizur was recently approved by the FDA for treatment of acquired hemophilia A and is similar enough to human blood to be effective in clotting, but less likely to be affected by autoantibodies.
In addition, her type and screen was positive for red blood cell antibodies and cross-matched blood would not be readily available if needed emergently. Our discussion with the surgeon included the likelihood of massive hemorrhage and alternative plans to reduce intraoperative blood loss requiring transfusion. Use of the Cell Saver blood-scavenging device was also requested.
The patient was induced with propofol, esmolol, and rocuronium; endotracheal tube intubation was uneventful. An arterial line was placed under ultrasound guidance to reduce the risk of hematoma formation from repeated attempts. Additional access included two supplementary 16-gauge peripheral IVs. The principle products available for acute bleeding included recombinant factor VII (Novoseven) and pro-thrombin complex concentrates (Kcentra), which are both available at our institution. In contrast to inherited factor VIII deficiency, desmopressin and cryoprecipitate are ineffective at treating acute bleeding in acquired forms. Fortunately, the intraoperative course did not consist of any major bleeding episodes and the patient was extubated at the end of the procedure. She recovered well in the PACU and was discharged home within 3-4 days.
CONCLUSIONS: Acquired hemophilia is an extremely rare, but potentially life-threatening, disorder affecting only 2 per 1 million people worldwide. This disorder does not respond to the standard treatments utilized for the inherited form. Preoperative planning should consist of a multi-disciplinary team approach comprising the surgeon, anesthesiologist, and hematologist. Factor VIII levels should be optimized prior to elective surgery and a plan to minimize and treat intraoperative blood loss is critical.