Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder caused by circulating autoantibodies (inhibitor) directed against coagulation factor VIII (FVIII). We report a 39-year-old single female who presented to emergency department with sudden onset gross hematuria 10 days following her first dose of Pfizer-BioNTech severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mRNA (coronavirus disease 2019 (COVID-19)) vaccine. Coagulation profile revealed isolated prolongation of the activated partial thromboplastin time due to FVIII deficiency with normal von Willebrand factor and activity. Mixing study revealed time-dependent inhibitor pattern that was successively identified as directed against FVIII using the Nijmegen-modified Bethesda assay. FVIII inhibitor in a titer of 17.2 Bethesda Units/mL was detected. While thrombosis is a frequent complication of severe COVID-19 infection, on the other hand, bleeding is rare in the setting of COVID-19 infection/vaccination with no anticoagulants. Till date, a couple of cases of acquired hemophilia developed after receiving mRNA derived COVID-19 vaccines (Pfizer-BioNTech SARS-CoV-2 mRNA vaccine and Moderna mRNA vaccines) had been reported. It is important to raise the awareness about this rare side effect that might be directly induced by the mRNA COVID-19 vaccine or that the vaccine could have triggered it in a genetically predisposed individual. We recommend considering screening for an inhibitor (by mixing study) in cases with otherwise unexplained onset hemorrhagic disorder and/or isolated activated partial thromboplastin time prolongation.
Keywords: Acquired hemophilia A; COVID-19 vaccine; Hemorrhage; Immune dysregulation.
Copyright 2022, Soliman et al.