Total knee arthroplasty in a female with a severe form of hemophilia A

Introduction. Hemophilia is an X-linked hereditary blood clotting disorder caused by insufficiency of factor VIII or IX that affects mainly men. In extremely rare cases, the disease can be observed in women, which most often associated with asymmetric inactivation X chromosome. The severity hemophilia women does not differ from Aim – to present a clinical observation surgical treatment stage 4 hemophilic arthropathy woman severe A. Main findings. Female patient T., 39 years old, was admitted National Medical Research Center for Hematology preliminary diagnosis: deficiency VIII. She had extension APTT 68.8 sec, high level Willebrand activity — 222 %, and concentration antigen 178.1 mg/l, decrease 1.6 %. According molecular genetic analysis, intron 22 inversion A detected F8 gene. Throughout patient’s life, hemarthrosis knee, ankle elbow joints were observed, led development varying severity. underwent total knee arthroplasty arthrolysis left joint. postoperative period proceeded without complications. Hemostatic replacement therapy performed recombinant