Background: Acquired haemophilia is an rare hemorrhagic disorders

Aims: We are representing the case of acquired haemophilia A and analyze the timeliness and adequacy of treatment.

Methods: The subject of the study was acquired haemophilia A

Results: Female, 33 years old, pregnancy II, urgent delivery. There was excessive bleeding in the early postpartum period, hemostatic therapy was provided. She hospitalized three times with recurrences of uterine bleeding. Vacuum aspiration of the walls of the uterine cavity was performed twice. After the third recurrence,  laparotomy and extirpation of the uterus was performed. Despite intensive care, the bleeding continued. Three relaparotomies were performed, the source of bleeding was not found, there was excessive bleeding from the operating field. The total blood loss was 24 447 ml. Intensive infusion-transfusion therapy was continued, which gave unstable hemostasis. The patient received 12 060 ml of fresh-frozen plasma, 15 130 ml of erythrocytes, 600 ml of albumin 10%, 20 doses of cryoprecipitate, 16 000 U of APCC, 8 mg of rVIIa, 8 000 U of PCC. 16,1 BU/ml of inhibitor FVIII, FVIII <1,0%, APTT – 146,1 s was detected on day 37. Within 30 days, blood loss was 10 608 ml; patient received 23 420 ml of fresh-frozen plasma, 2 080 ml of erythrocytes, 17 mg of rVIIa, 19 000 U of   APCC, 6 000 U of PCC, 61 dose of cryoprecipitate, 50 000 IU of FVIII. Immunosuppressive therapy (prednisolone 1-1,5 mg/kg/day) was started on day 67 and lasted for 12 months. Bleeding stopped completely 10 months after delivery, and after 14 months the inhibitor was not detected, factor levels and APTT returned to normal.

Conclusions: Recurrent postpartum hemorrhage requires early diagnosis of coagulation factors inhibitors, which will ensure specific transfusion and immunosuppressive therapy, avoidance of serious consequences, including extirpation of the uterus and reduce the economic costs of treatment.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/the-case-of-acquired-haemophilia-a/