Abstract Number: PB0926
Meeting: ISTH 2020 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical
Background: The development of inhibitors (antibodies against FVIII/FIX concentrates) is the main complication in the treatment of hemophilia. Hemophilia patients with inhibitors are more vulnerable to arthropathy and orthopedic and musculoskeletal complications leading to a prolonged hospital stay and uncontrolled bleeding.
Aims: In this study, we evaluated our hemophilic patients with inhibitor during orthopedic surgery.
Methods: We reviewed a series of orthopedic surgery procedures in hemophilia patients with inhibitors who underwent between 2019 and 2018 at our hospital.
In total, 5 surgeries were conducted in 4 hemophilia patients with inhibitors. The median age of the patients was 10.5 (range, 6-16) years. Our clinical series of patients comprised 3 cases of hemophilia A (75%) and 1 cases of hemophilia B (25%). All of the patients had severe hemophilia, with factor activity in blood less than 1%. The inhibitor titer at procedures was range – 3,5 – 562 BU. Elective surgical procedures were performed 1 achilloplasty in patient with equinus position of the left foot, 1 arthroscopic debridement of left knee, 1 internal fixation for femur fracture and 1 removing metal fixation, 1chemical synovectomy with rifampicin of left knee in patient with inhibitory hemophilia B. In total, 2 patients received rFVIIa, 2 patients – rFVIIa and APCC.
Results: In most cases, postoperative bleeding control was successful. However, 1 patient with inhibitory hemophilia B during the last procedure of rifampicin injection had acute hemarthrosis of left knee, which was controlled by 3-day infusions of rFVIIa; 1 patients after arthroscopic debridement without appropriate rehabilitation has progression of hemophilic arthropathy of left knee.
Conclusions: Hemophilia patients with inhibitor present difficult management challenges when they need surgery. Surgery in patients with inhibitors requires more careful preparation and efficiency depend on not only hemostatic agents, but on organisation of multidisciplinary team (surgeons, hematologist and physiotherapist) in the comprehensive hemophilia treatment centers.
To cite this abstract in AMA style:
Kashpurenko Y, Vilchevska K, Bakhchyvandzhy I, Vitiaz V. The Experience of Orthopedic Surgeries in Ukrainian Hemophilia Patients with Inhibitor [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/the-experience-of-orthopedic-surgeries-in-ukrainian-hemophilia-patients-with-inhibitor/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/the-experience-of-orthopedic-surgeries-in-ukrainian-hemophilia-patients-with-inhibitor/