Abstract Number: PO147
Meeting: ISTH 2021 Congress
Background: Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII).
Identified on patients with no previous personal or family history of bleeding and an isolated prolonged activated partial thromboplastin time (aPTT).
Prompt diagnosis of this disorder is essential to control hemorrhage and suppress the inhibitor appropriately.
Aims: Describing bleeding control in a patient with AHA.
Methods: Review a clinical case.
Results: A 66‐year‐old man, previously healthy and no history of bleeding, was submitted to a laparoscopic cholecystectomy, complicated by peritonitis after iatrogenic small bowel perforation.
Six months later, he had an abdominal wall abscess with enterocutaneous fistula. Abscess drainage and later bowel transit reconstruction were performed.
Ten days postoperatively, he presented anastomosis dehiscence and intra-abdominal hemorrhage, with necessity of hematoma drainage and abdominal packing for uncontrollable bleeding.
Screening coagulation tests showed a prolonged aPTT (67.9″), a normal prothrombin time and fibrinogen.
A mixing study resulted in failure of aPTT correction (Rosner index: 18%), suggesting the presence of a factor inhibitor. Factors levels: FVIII 17%, FIX 139.5%, FXI 62.9%.
AHA etiological investigation started.
The patient was initially treated with plasma-derived coagulation FVIII concentrate due to unavailability of alternative therapy and initiated corticosteroid therapy. In D2, the packing was removed and 4 draining tubes were inserted under recombinant activated factor VII (90 μg/kg every 3 hours) and transfusion support, with significant improvement of the hemorrhagic episode. In D12, he switched to anti-inhibitor coagulant complex (FEIBA).
Although the hemorrhagic episodes were successfully controlled, and the inhibitor title lowered, the patient had gradual health status deterioration by the infectious condition dying 19 days after the diagnosis of AHA.
Conclusions: Identifying the etiology of AHA and treating underlying co‐morbidities, as well as controlling bleeding and infections associated to immunosuppression, are key to reduce morbidity and mortality in AHA.
To cite this abstract in AMA style:Cabral J, Afonso M, Costa L, Malheiro B, Calheiros M, Barbosa AP, Calaza C, Majar M, Carvalho G, Marques A. Management of Acquired Hemophilia A: A Case-report [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/management-of-acquired-hemophilia-a-a-case-report/. Accessed June 25, 2022.
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