Acquired Hemophilia A after SARS-CoV-2 Infection: A Case Report and an Updated Systematic Review

Abstract

The role of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) has been implicated in the pathogenesis of acquired hemophilia A (AHA). The aim of this study is to report our case and to summarize clinical studies on de novo AHA after SARS-CoV-2 infection. We performed a systematic search on the association of SARS-CoV-2 with AHA in four medical databases up to 28 May 2023. Eligible studies should include de novo AHA patients who had SARS-CoV-2 infection before or concomitant with the diagnosis of AHA. Findings were synthesized narratively. In addition, we report the case of a 62-year-old female patient, who presented to our clinic with left flank pain 2 weeks after SARS-CoV-2 infection. Clinical investigations confirmed AHA and imaging studies revealed retroperitoneal bleeding. Her hemostasis was successfully secured with bypassing agents; however, despite immunosuppressive therapy, high inhibitor titer persisted. In the systematic review, we identified only 12 relevant cases with a questionable cause-effect relationship between SARS-CoV-2 infection and AHA. Based on the qualitative analysis of the relevant publications, current clinical evidence is insufficient to support a cause-effect relationship. The analysis of data from ongoing AHA registries can serve further evidence.

Keywords: COVID-19; SARS-CoV-2; acquired hemophilia; case report; coagulopathy; hemostasis.

Figure 1

Abdominal CT angiography. CT scans revealed a retroperitoneal hematoma with an extension of 9 × 20 cm at the psoas muscle on the left side. The red arrow shows the retroperitoneal hematoma.

Figure 2

ClotPro results. The bedside test was performed post-operatively immediately on intensive care unit admission. The EX–test yielded normal coagulation time with an acceptable clot formation without any considerable fibrinolysis, whereas the IN-test shows no coagulation.

Figure 3

Timeline. Two weeks prior to hospital admission, the patient had SARS-CoV-2 infection. At the current emergency room (ER) presentation, the patient already had a negative test for SARS-CoV-2 virus infection. After surgical evacuation of the retroperitoneal hematoma, the patient was transferred to the intensive care unit, where hemostasis was supported by recombinant activated factor VII, and autoantibody eradication was treated with immunosuppressive therapy and plasmapheresis. The surgical hematoma was evacuated, and surgical swabs were regularly replaced. ER: emergency department, FEIBA: factor VIII inhibitor bypass activity, ICU: intensive care unit, rFVIIa: recombinant activated factor VII.