Total Hip Replacement in a Hemophilia-a Patient with High Titer Inhibitor
Authors: Kaya Zühre , Orhan Özlem , Turanli Sacit , Gürsel Türkiz , Kocak Ulker , Yenicesu Idil 
Published: October 2019

Abstract
BACKGROUND
Patients with hemophilia seldom need joint arthroplasty today, due to decrease of bone and cartilage damage under factor prophylaxis started early childhood. However, hemophilia patients with inhibitors often develop severe joint damage as a result of ongoing bleedings. It is a formidable challenge to manage post-operative bleedings with bypassing agents in such cases. There have been only a few reports of major hip surgery in hemophilia A with inhibitors. Here we report a case of successful total hip replacement in an 18 year old adolescent boy with high titer inhibitor since infancy under recombinant factor VIIa (rVIIa) coverage.
PATIENT
The patient has been followed up with high titer inhibitor ranging between 35- 95 BU since he was three years old and received mostly on demand treatment with either rVIIa (NovoSeven®) or activated prothrombin complex concentrate (APCC) (FEIBA®). Prophylaxis with APCC and rVIIa were also given for 3 years and 6 months, respectively. During the last two years, he experienced frequent bleedings in his right hip joint and developed crippling joint damage and assessed as grade IV according to the World Federation of Hemophilia grading system leading to severe chronic pain requiring opiate analgesics, shortened right leg and difficulty in walking and climbing. Preoperative inhibitor titer was 64 BU. Intravenous rVIIa was given in a dose of 270 mcg/kg, 30 minutes before surgery, repeated in 120 mcg/kg dose two hours after the first dose and continued in 90-120 mcg/kg doses during the first 24 hours. Intraoperative hemostasis was excellent and only two units of red cell transfusion was needed. However, he developed severe bleedings at the surgery site in the second day of surgery, which was partially controlled with 270 mcg/kg rVIIa. Four units of red cell transfusion was given and tranexamic acid 4x1 g daily was started. Since 120 mcg/kg rVIIa in every 2-3 hours did not stop bleeding, we added 50IU/kg APCC twice daily, rVIIa was reduced to 90 mcg/kg and tranexamic acid was stopped. No thromboprophlaxis was given. Although bleeding gradually decreased eventually over the next 3 days, he developed high fever with increased CRP level and re-bleeding at the surgery site at the fifth day suggesting wound infection. Broad spectrum antibiotics combined with bypassing agents was continued for 21 days during which no bleeding was noted. At 1 month, he had excellent joint function and clear surgery site. He is still receiving only APCC prophylaxis 50 U/kg three days weekly. Treatment cost was calculated as postoperative changes in rVIIa and APCC doses related to the size of vials and patient weights. Total amount of treatment cost was approximately 610.000 $ US for rVIIa and 66.000 $ US for APCC during hospitalization.
CONCLUSIONS
Total hip replacement is rarely performed in young hemophilia patients with high titer inhibitor due to difficulty in management of bleedings and high cost of treatment product. However, it can be unavoidable in severely crippled patients, incapable of doing daily activities and suffering from intense pain. Our experience in the present case is encouraging for performing hip arthroplasty in such cases. No excessive bleeding occurred during surgery under rVIIa treatment. Combined use of rVIIa and APCC for secondary bleedings during post-operative period is safe and effective. High cost of surgery emphasize the need for development of suitable prophylaxis regimes in children with inhibitor to prevent crippling arthropathy.

Disclosures
No relevant conflicts of interest to declare.