Treatment tactics for a patient with acquired hemophilia: continuous infusion of recombinant activated coagulation factor VII and the inhibitor eradication
نویسندگان
چکیده
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 and women often associated with pregnancy. Aim — present case successful treatment patient acquired using long-term continuous infusion rFVIIa eradication as result immunosuppressive therapy. Main findings. A clinical observation severe hemorrhagic syndrome pregnancy presented. Uterine bleeding after spontaneous delivery, refractory standard treatment, required multiple surgical interventions. Laboratory tests showed prolongation APTT, CT INTEM rotational thromboelastometry test, decrease plasma FVIII activity presence FVIII. Vacuum-assisted closure was applied treat infected laparotomy wound. Therapy for consisted hemostatic therapy eradication. Hemostatic included at rate 30 μg/kg/h gradual up 9.6 μg/kg/h, well its fractional administration before every surgery dosage 80 μg/kg. The effectiveness assessed dynamics test shortening APTT. achieved prednisolone therapy, combined immunosuppression rituximab azathioprine, followed replacement cyclophosphamide. As result, hemorrhage stopped, reference were reached.
منابع مشابه
Treatment of an acquired coagulopathy with recombinant activated factor VII in a damage-control patient.
Recombinant activated factor VII is commonly used for the treatment of hemophiliac patients with inhibitors and has been studied for use in trauma. We report the use of recombinant activated factor VII for a male patient who was injured in a motor vehicle accident. We also summarize the animal studies and clinical trials that have been reported.
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Abstract Nowadays, bypassing agents such as recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCC) are used to treat bleeding episodes in the Hemophilia patients with inhibitors. AryoSeven® is an Iranian biogeneric rFVIIa with homogeneity of efficacy and the nature to NovoSeven in a comparative trial. The current clinical trial aimed to evaluate the cost...
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Two-incision laparoscopic appendectomy for a severe hemophilia A child patient with coagulation factor VII deficiency
RATIONALE The main complication of patients with severe hemophilia is recurrent bleeding events that usually affected musculoskeletal contractures. And replacement therapy methods were continuously improved to minimize adverse impacts brought by those complications. However, only several cases reported about the appendectomy for hemophilia A. We report a case of acute appendicitis treated by tw...
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ژورنال
عنوان ژورنال: Gematologiia i transfuziologiia
سال: 2022
ISSN: ['0234-5730', '2411-3042']
DOI: https://doi.org/10.35754/0234-5730-2022-67-2-282-294