Introduction. Acquired hemophilia is a rare autoimmune disease caused by an inhibitor to clotting factor VIII (FVIII). It complicates the course of many diseases, in particular autoimmune diseases, and in women is often associated with pregnancy.Aim — to present a case of successful treatment of a patient with acquired hemophilia using long-term continuous infusion of rFVIIa and inhibitor eradication as a result of immunosuppressive therapy.Main findings. A clinical observation of severe hemorrhagic syndrome in a patient with acquired hemophilia associated with pregnancy is presented. Uterine bleeding in the patient after spontaneous delivery, refractory to standard hemorrhagic syndrome treatment, required multiple surgical interventions. Laboratory tests showed prolongation of APTT, CT in the INTEM rotational thromboelastometry test, a decrease in plasma FVIII activity and presence of inhibitor to FVIII. Vacuum-assisted closure was applied to treat infected laparotomy wound. Therapy for acquired hemophilia consisted of hemostatic therapy and the inhibitor eradication. Hemostatic therapy included a continuous infusion of rFVIIa at a rate of 30 μg/kg/h with a gradual decrease up to 9.6 μg/kg/h, as well as its fractional administration before every surgery at a dosage of 80 μg/kg. The effectiveness of the therapy was assessed by the dynamics of CT in the INTEM test and the shortening of the APTT. The inhibitor eradication was achieved by prednisolone therapy, combined immunosuppression with rituximab and azathioprine, followed by its replacement with cyclophosphamide. As a result, the hemorrhage was stopped, reference plasma activity of FVIII and eradication of the inhibitor were reached.

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

Introduction. In 2018 emicizumab was approved in Russia for prophylactic treatment in patients with hemophilia A (HA) with inhibitors and in 2019 for patients with severe HA without inhibitors. A significant amount of data has been accumulated from clinical trials and real-world data, which allow us to resolve most of the questions that hematologists may have when to prescribe emicizumab.Aim — to provide information on the management of patients on emicizumab.Results. The recommendations accumulated the currently available information and world experience in the management of patients receiving emicizumab in order to facilitate decision-making when prescribing and using emicizumab. Information on the use of emicizumab in patients with HA with FVIII inhibitors and severe HA without FVIII inhibitors is presented. Possible complications and measures for their prevention and treatment are presented.