COVID-19 in Patients with Acquired Hemophilia A − Case Reports
At the XXVIII. Pařízek Days held in Ostrava, on the afternoon of March 17, 2022, Dr. Anna Maria Pejková from the 4th Internal Clinic of the University Hospital Hradec Králové presented 3 case reports of acquired hemophilia A.
Possible Link Between AHA and COVID-19
Acquired hemophilia A (AHA) is a rare autoimmune disease with an incidence of approximately 1.5 per 1,000,000 people. The risk group primarily consists of individuals over 60 years old, with about half presenting with life-threatening bleeding. In the last approximately 1.5 years, there have been more newly diagnosed cases of AHA in the Hradec Králové region than in previous years.
COVID-19 infection, among other things, leads to immune system dysregulation with the risk of autoreactive responses. According to some studies, there is an observed increase in the production of certain autoantibodies (ANA, ANCA) in connection with the infection.
Case Report 1
The first presented case concerned a 74-year-old woman with a history of advanced chronic obstructive pulmonary disease (COPD). She presented in July 2021 with melena and anemia, along with extensive skin hematomas. Her medical history includes prolonged bleeding after the extraction of several teeth. The woman had been vaccinated with the second dose of the COVID-19 vaccine (AstraZeneca) approximately one month prior.
Based on tests, her coagulation factor VIII (FVIII) level was determined to be 5.8%, and the presence of an FVIII inhibitor (titer 1.4 BU) was confirmed. Hemostatic treatment was initiated with a bypassing agent (rFVIIa), and intravenous corticosteroids were administered to eradicate the inhibitor. During treatment, antibiotics for pneumonia and antifungals for extensive oral thrush were administered. The eradication of the inhibitor was successful, at the time of discharge, the patient was in remission (FVIII 166%), and was sent home with a small dose of prednisone. Remission persisted for 2 months post-discharge.
Case Report 2
The next patient was an 82-year-old woman with dementia, admitted for investigation in April 2021 due to the development of spontaneous hematomas and anemia. Her history also included COVID-19 vaccination (Pfizer/BioNTech) approximately one month before the first symptoms of AHA.
The initial FVIII level was 1%, and an FVIII inhibitor titer of 64.2 BU was confirmed. Hemostatic control was achieved with a bypassing agent (rFVIIa), and eradication of the inhibitor was attempted with a combination of corticosteroids and cyclophosphamide. The patient achieved disease remission and was discharged with a small dose of corticosteroids to home care, with remission persisting 4 months post-discharge.
Case Report 3
In the last, very interesting case report, a 78-year-old man with long-term diagnosed congenital hemophilia A with a basal FVIII level around 4% was mentioned. He presented in June 2021 with bleeding symptoms and anemia. Recent medical history included COVID-19 vaccination with the Moderna vaccine.
The administered FVIII concentrate was ineffective, initial laboratory tests showed an FVIII level of 1%, and a low titer FVIII inhibitor (1.3 BU) was confirmed. Corticosteroids were administered, and remission was subsequently confirmed.
Discussion and Conclusion
According to the presenter, these case reports share the higher age of the patients, existing comorbidities, negative oncological history, and significant skin bleeding diathesis. All three cases also had preceding COVID-19 vaccination, involving three different vaccines. The median AHA development from the vaccine administration was 26 days. In all cases, disease remission was achieved.
Similar cases have also been published in the professional literature as case reports or in small cohorts. It remains a question whether this suggests a possible influence of COVID-19 vaccination in the elderly on the development of this otherwise rare disease or a random coincidence (especially considering that the senior population is among the most vaccinated groups). It is also crucial to emphasize that the benefits of vaccination in the general population significantly outweigh these rather hypothetical risks.
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Source: Pejková A. M. Acquired Hemophilia and COVID-19. XXVIII. Pařízek Days, Ostrava, March 17, 2022.
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