Acute-type acquired hemophilia A after COVID-19 mRNA vaccine administration: A new disease entity?

Hosoi, Hiroki; Tane, Misato; Kosako, Hideki; Ibe, Masaki; Takeyama, Masahiro; Murata, Shogo; Mushino, Toshiki; Sawada, Takashi

doi:10.1016/j.jaut.2022.102915

Cited by 7 publications

(6 citation statements)

References 35 publications

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“…The reason why our patient developed AHA is not clear. In the last few years, cases of AHA in COVID patients have been described, both after infection and vaccination [15][16][17][18][19][20][21][22][23][24][25]. A few cases of AHA following the viral infection have been reported in literature [15,[26][27][28][29][30].…”

Section: Discussionmentioning

confidence: 99%

Hematological Complications in a COVID-19 Patient: A Case Report

Ianuà,

Caldarelli,

De Matteis

et al. 2023

Diseases

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Hemophilia A is a hemorrhagic disorder caused by insufficient or inadequate coagulation factor VIII activity. Two different forms are described: congenital, hereditary X-linked, and acquired. Acquired hemophilia A (AHA) is a rare condition and it is defined by the production of autoantibodies neutralizing factor VIII, known as inhibitors. We report the case of a 72-year-old man with a clinical diagnosis of AHA after SARS-CoV-2 infection, which has been described in association with several hematological complications. SARS-CoV-2 infection could represent the immunological trigger for the development of autoantibodies. In our patient, SARS-CoV-2 infection preceded the hemorrhagic complications by 15 days. This lag time is in line with the other cases reported and compatible with the development of an intense immune response with autoantibody production. It is possible that since our patient was affected by type 1 diabetes mellitus, he was more prone to an immune system pathological response against self-antigens. A prompt, appropriate therapeutic intervention with activated recombinant factor VII administration and cyclophosphamide has led to rapid remission of clinical and laboratory findings.

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Section: Discussionmentioning

confidence: 99%

Hematological Complications in a COVID-19 Patient: A Case Report

Ianuà,

Caldarelli,

De Matteis

et al. 2023

Diseases

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“…However, there are some reports of patients with acquired hemophilia A following COVID-19 vaccination [38][39][40][41][42][43][44]. Duminuco et al described this rare coagulopathy associated with hemorrhagic complications due to the possible development of FVIII inhibitors following an immune stimulus [38].…”

Section: Inherited Blood Coagulation Disorders and Covid-19 Vaccinesmentioning

confidence: 99%

Blood Coagulation and Thrombotic Disorders following SARS-CoV-2 Infection and COVID-19 Vaccination

Sekulovski,

Mileva,

Vasilev

et al. 2023

Biomedicines

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Although abundant data confirm the efficacy and safety profile of the developed vaccines against COVID-19, there are still some concerns regarding vaccination in high-risk populations. This is especially valid for patients susceptible to thrombotic or bleeding events and hesitant people due to the fear of thrombotic incidents following vaccination. This narrative review focuses on various inherited and acquired thrombotic and coagulation disorders and the possible pathophysiologic mechanisms interacting with the coagulation system during immunization in view of the currently available safety data regarding COVID-19 vaccines. Inherited blood coagulation disorders and inherited thrombotic disorders in the light of COVID-19, as well as blood coagulation and thrombotic disorders and bleeding complications following COVID-19 vaccines, along with the possible pathogenesis hypotheses, therapeutic interventions, and imaging for diagnosing are discussed in detail. Lastly, the lack of causality between the bleeding and thrombotic events and COVID-19 vaccines is debated, but still emphasizes the importance of vaccination against COVID-19, outweighing the minimal risk of potential rare adverse events associated with coagulation.

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“…A specific mention deserves attention, being recently identified acquired hemophilia A following coronavirus disease 2019 (COVID-19) vaccination. Although a causality link between vaccine and acquired hemophilia A has not been definitely ascertained, several of such cases have already been reported [ 29 , 30 , 31 , 32 , 33 ]. A possible explanation is that vaccines may foster or boost immune reactions, followed by insurgence of new autoimmune events or exacerbation of pre-existing but latent immune dysregulations, which can ultimately drive the clonal selection of B cells producing specific autoantibodies, including those against FVIII.…”

Section: Autoimmune Disorders Leading To Bleedingmentioning

confidence: 99%

“…For example, one recent case-based review identified 16 cases associated with inflammatory arthritis, SLE, vasculitis, Jo1 syndrome, Guillain-Barré syndrome, myocarditis [ 107 ]. Happaerts et al described a case of acquired hemophilia A post vaccination, and reviewed 17 other cases reported in the literature [ 31 ]. Of interest, unlike VITT, which is associated with the use of adenovirus vaccines, most cases of acquired hemophilia A have been associated with mRNA-based vaccines (Pfizer [BNT162b2/Comirnaty] and Moderna [mRNA-1273/Spikevax]) [ 29 ].…”

Section: Autoantibodies Interfering With Hemostasis In Covid-19 and A...mentioning

confidence: 99%

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

Favaloro

Pasalic

Lippi

2022

IJMS

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Hemostasis reflects a homeostatic mechanism that aims to balance out pro-coagulant and anti-coagulant forces to maintain blood flow within the circulation. Simplistically, a relative excess of procoagulant forces can lead to thrombosis, and a relative excess of anticoagulant forces can lead to bleeding. There are a wide variety of congenital disorders associated with bleeding or thrombosis. In addition, there exist a vast array of autoimmune diseases that can also lead to either bleeding or thrombosis. For example, autoantibodies generated against clotting factors can lead to bleeding, of which acquired hemophilia A is the most common. As another example, autoimmune-mediated antibodies against phospholipids can generate a prothrombotic milieu in a condition known as antiphospholipid (antibody) syndrome (APS). Moreover, there exist various autoimmunity promoting environments that can lead to a variety of antibodies that affect hemostasis. Coronavirus disease 2019 (COVID-19) represents perhaps the contemporary example of such a state, with potential development of a kaleidoscope of such antibodies that primarily drive thrombosis, but may also lead to bleeding on rarer occasions. We provide here a narrative review to discuss the interaction between various autoimmune diseases and hemostasis.

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Acute-type acquired hemophilia A after COVID-19 mRNA vaccine administration: A new disease entity?

Cited by 7 publications

References 35 publications

Hematological Complications in a COVID-19 Patient: A Case Report

Hematological Complications in a COVID-19 Patient: A Case Report

Blood Coagulation and Thrombotic Disorders following SARS-CoV-2 Infection and COVID-19 Vaccination

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

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