Acquired Hemophilia a in the Modern Medical Reality: Practical Clinical Experience

Abstract. Introduction. Acquired hemophilia A is a rare form of severe coagulopathy with an autoimmune mechanism of development. It occurs due to the formation of circulating inhibitory factor VIII (FVIII) antibodies, which leads to impaired activation of factor X and the occurrence of spontaneous bleeding in patients without previous hemostasis system pathology. In about a half of the cases, FVIII autoantibodies appear in patients without any comorbidities. It should be noted that the average age of patients was 74 years at the time of diagnosis. However, the occurrence of the disease in young women is rare and may be associated with pregnancy and childbirth. Given the rarity of this disease, the management of these patients is a challenging clinical problem. Aim. To describe a clinical case of diagnosis and treatment of a patient with the inhibitor form of acquired hemophilia A (AHA). Materials and Methods. A 38–year–old female patient had acute hemorrhagic syndrome of the hematoma type, manifested by large hematomas in different areas of the body. During the patient’s stay in the hospital, standard lab tests were made, the test results were evaluated over time, and instrumental diagnostics was performed. Results and Discussion. The typical hematoma type of bleeding detected during diagnosing, prolongation of coagulation parameters, presence of factor VIII inhibitor (605 BU), and a decrease in factor VIII level down to 0.5%) verified the AHA diagnosis. Methods of substitution, immunosuppressive therapy, and monoclonal antibodies were successfully used to treat AHA in this patient. The patient is followed up by a hematologist at the place of residence. Hematomas did not recur. Conclusions. This clinical observation demonstrates the peculiarities of the course, diagnostic algorithm, and management of patients with AHA without any concomitant pathology, with a history of only prolonged postpartum uterine bleeding, and with no autoimmune or malignant diseases.