title: Successful management of total knee replacement in a high responder hemophilia patient with a history of inhibitor.
abstract: The development of inhibitors against administered clotting factors may render replacement therapy ineffective for some hemophilia patients. Such patients are therefore at the highest risk of developing arthropathy. Elective orthopedic surgery (EOS) in hemophilic patients having such inhibitors remains a rare, expensive, and difficult surgery, whose management represents a significant challenge. We report the case of a 35-year-old man with a severe form of hemophilia A (factor VIII < 1%), who was suffering from repetitive spontaneous hemarthrosis, especially in his knee joints that had consequently become more susceptible to bleeding. The patient had a history of high levels of factor VIII inhibitor (> 5.0 Bethesda Unit [BU]/ml) as shown by the factor VIII inhibitor assay; therefore, we began treatment with factor VIIa for his mild-to-moderate bleeding (90 µg/kg intravenous bolus injections). The interval between injections varied with the severity of the hemorrhage in each bleeding episode. The inhibitor level reduced to 3.1 BU/ml after three months, to 1.6 BU/ml after six months, and disappeared completely after one year of treatment. We administered factor VIII at a dose of 50 IU/kg every eight hours during the first three post-operative days, then continued administration with a dose of 40 IU/kg every 12 hours for another four days, and observed a very good response to treatment with no bleeding. Recombinant activated factor VII (rFVIIa) is not an inhibitor-removal strategy, but an inhibitor-bypassing product. However, in our patient, the treatment of mild-to-moderate bleeding with short-term use of rFVIIa and no exposure to factor VIII caused a gradual reduction in the inhibitor level over a period of 1 year.
authors.affiliation: Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Hemophilia and Thalassemia Department, Tabriz, IR Iran.
authors.forename: Roya
authors.initials: R
authors.lastname: Dolatkhah
authors.forename: Mohammad Reza
authors.initials: MR
authors.lastname: Bazavar
authors.forename: Masoud
authors.initials: M
authors.lastname: Poureisa
authors.forename: Iraj
authors.initials: I
authors.lastname: Asvadi Kermani
authors.forename: Jalil
authors.initials: J
authors.lastname: Vaez Gharamaleki
authors.forename: Zohreh
authors.initials: Z
authors.lastname: Sanaat
authors.forename: Jamal
authors.initials: J
authors.lastname: Eivazi Ziaei
authors.forename: Alireza
authors.initials: A
authors.lastname: Nikanfar
authors.forename: Ali
authors.initials: A
authors.lastname: Esfahani
authors.forename: Seyed Hadi
authors.initials: SH
authors.lastname: Chavoshi
keywords: Hemophilia A; Hemophilia B; Inhibitor; Orthopedics