ABSTRACT
Background : Acquired hemophilia A (AHA) is caused by autoantibodies against coagulation factor VIII (FVIII). Treatment focuses on both eradicating the inhibitor and treating and preventing bleeding episodes. SARS-CoV-2 infection causes a high mortality hipercoagulable state. Aims : Review AHA case with high risk of bleeding and thrombotic complications. Methods : We present a patient with refractory AHA treated with immunosupression, SARS-CoV-2 infection and iliopsoas hematoma treated with recombinant porcine FVIII (rpFVIII). Results : 73 year old male was admitted because of ecchymosis, hematuria and anemia (Hb 8.5 g/dL). Coagulation tests showed APTT ratio 2.12, FVIII:C 1.8 UI/dL and anti-FVIII 156 BU. He was diagnosed with AHA and put on rFVIIa, prednisone and rituximab. Three weeks later he developed severe respiratory insuffiency, bilateral pneumonia and severe acute immflamatory response with a positive PCR test for SARS-CoV-2. Last dose of rituximab was postponed and treatment with metilprednisolone, tocilizumab, lopinavir/ritonavir, hidroxicloroquine and prophylactic heparin (LMWH) was started. He was enrolled on a clinical trial with remdesivir. 4 days later he related acute pain on the right hip with inability to walk. CT scan showed iliopsoas hematoma. Due to high thrombotic risk of using bypass agents, a request for rpFVIII (susoctocog alfa -Obizur®) was made. After SARS-CoV2 infection was resolved, treatment with mofetil mycophenolate was started (no response to previous treatment). Inhibitor title is <0.4 B. FVIII levels remain above 50 UI/dL Conclusions : Patients with AHA and SARS-CoV-2 infection are at high risk of bleeding and thrombotic events, complicating treatment with bypass agents. RpFVIII, allows to extend time between infusions, to monitor levels of FVIII. It is a good option to treat bleeding episodes without adding thrombotic risk. After tocilizumab administration a reduction on the inhibitor title was seen. It may be have a roll in refractory AHA. The use of LMWH is not well stablished for patients with Covid19 and AHA. Conclusions : Acquired postpartum hemophilia A represents the most frequent etiology in our series;the time to onset is very variable, ranging from the immediate postpartum up to 4 months.