Acquired hemophilia A (AHA) due to anti‐SARS‐CoV‐2 vaccination: A systematic review

Amisha, Fnu; Saluja, Prachi; Malik, Paras; Rhee, Frits van

doi:10.1002/jha2.604

Cited by 4 publications

(3 citation statements)

References 37 publications

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“…Both molecular mimicry and activation of dormant lymphocytes specific to factor VIII can lead to the production of autoantibodies. [ 22 ] In our analysis of 17 case series, we did not find a significant difference between the first and second vaccination or between the 2 types of vaccine products.…”

Section: Discussionmentioning

confidence: 60%

Severe acquired hemophilia A associated with COVID-19 vaccination: A case report and literature review

Kim,

Jung,

Lee

et al. 2024

Medicine

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Rationale: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by an antibody that inhibits coagulation factor VIII activity. More than half of patients with AHA cannot identify underlying disorders. The remaining patients are associated with malignancies, autoimmune diseases, skin diseases, infections, and medications. Here, we present a case of 56-year-old Korean man with underlying hypertension, dyslipidemia, and diabetes mellitus who developed AHA following the second dose of BNT162b2 COVID-19 vaccination. Patient concerns: He presented with a large 20 × 30 cm-sized hematoma along the psoas muscle and intracranial hemorrhage, necessitating intensive care with mechanical ventilation and continuous renal replacement therapy. Laboratory testing demonstrated that activated partial thromboplastin time and prothrombin times were 74.7 seconds (normal range 29–43 seconds) and 17.2 seconds (normal range 12.5–14.7 seconds), respectively. Diagnoses: Laboratory tests confirmed AHA with undetectable factor VIII activity (<1.5%) and a positive factor VIII antibody with a titer of 8.49 Bethesda units/mL. Interventions: Recombinant factor VIIa (NovoSeven®) was administered every 2 hours to control the bleeding, alongside immunosuppression with methylprednisolone 1 mg/kg daily and cyclophosphamide 2 mg/kg daily to eliminate the autoantibody. Outcomes: Despite the treatments, the patient developed sepsis and succumbed 14 weeks after admission. Lessons: This rare case underscores the importance of monitoring for AHA following COVID-19 vaccination. Although the benefits outweigh the risks of vaccination, AHA should be considered in the differential diagnosis of unusual bleeding following the vaccinations. Early diagnosis and management before severe bleeding are critical for successfully controlling life-threatening bleeding.

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Section: Discussionmentioning

confidence: 60%

Severe acquired hemophilia A associated with COVID-19 vaccination: A case report and literature review

Kim,

Jung,

Lee

et al. 2024

Medicine

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“…AHA occurs in both men and women, most commonly in elderly patients (>60 years old) and postpartum females. While AHA is often associated with other autoimmune diseases, malignancy, or medications, approximately 1/2 of the cases occur in healthy individuals 46–48 . Similar to congenital haemophilia complicated by inhibitors, overall management of AHA requires a multipronged approach: treatment of acute bleeding, bleed prophylaxis, and inhibitor eradication.…”

Section: Discussionmentioning

confidence: 99%

“…While AHA is often associated with other autoimmune diseases, malignancy, or medications, approximately 1/2 of the cases occur in healthy individuals. [46][47][48] Similar to congenital haemophilia complicated by inhibitors, overall management of AHA requires a multipronged approach: treatment of acute bleeding, bleed prophylaxis, and inhibitor eradication. Bleeding events in AHA are often serious and require prompt haemostatic management.…”

Section: 3mentioning

confidence: 99%

Haemophilia in the era of novel therapies: Where do inhibitors feature in the new landscape?

Meeks,

Zimowski

2024

Haemophilia

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IntroductionThe advent of therapeutic recombinant factor VIII (FVIII) and factor IX (FIX) protein infusions revolutionized the care of persons with haemophilia in the 1990s. It kicked off an era with the increasing use of prophylactic factor infusions for patients and transformed conversations around the ideal trough activity levels as well as the ultimate goals in tailored, individualized care. Our knowledge surrounding the immunologic basis of inhibitor development and treatment derives from a time when patients were receiving frequent factor infusions and focused on immune tolerance induction following inhibitor development.DiscussionMore recently, care was revolutionized again in haemophilia A with the approval of emicizumab, a bispecific antibody mimicking activated FVIII function, to prevent bleeding. The use of emicizumab prophylaxis has resulted in a significantly slower accumulation of factor exposure days and continued effective prophylaxis in the case of inhibitor development. While emicizumab is effective at reducing the frequency of bleeding events in patients with haemophilia A, management of breakthrough bleeds, trauma, and surgeries still requires additional treatment. Ensuring that FVIII is a therapeutic option, particularly for life‐threatening bleeding events and major surgeries is critical to optimizing the care of persons with haemophilia A. Other novel non‐factor concentrate therapies, including rebalancing agents, will dramatically change the landscape for persons with haemophilia B with inhibitors.ConclusionThis review discusses the changing landscape regarding the timing of inhibitor development and management strategies after inhibitor development, stressing the importance of education across the community to continue to vigilantly monitor for inhibitors and be prepared to treat persons with inhibitors.

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Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

Lehoczki,

Fekete,

Mikala

et al. 2024

GeroScience

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Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder characterized by the development of neutralizing autoantibodies (inhibitors) against coagulation factor VIII (FVIII). This review provides an in-depth exploration of AHA, covering its epidemiology, pathogenesis, clinical presentation, diagnosis, complications, and treatment strategies, focusing on recent advancements. AHA can manifest in both men and women with no prior bleeding history. The annual incidence is estimated to be approximately 1 case per million individuals in the general population. The incidence increases significantly with age: the incidence among individuals aged 60 years or older is approximately 3 to 4 cases per million individuals per year. Typically, patients present with an acquired bleeding disorder that is characterized by an isolated, prolonged activated partial thromboplastin time stemming from FVIII deficiency. Diagnosis relies on the detection of neutralizing antibodies using the Nijmegen-modified Bethesda assay. Hemostatic control strategies involve bypassing agents like recombinant activated factor VII, activated prothrombin complex concentrate, and recombinant porcine FVIII for bleeding patients. Emicizumab, a novel bypassing agent, exhibits several potential advantages. In the realm of immunosuppressive treatment for inhibitor eradication, the CyDRi regimen emerged as a remarkable advancement, significantly enhancing the outlook for the management of AHA even in the elderly frail population.

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Acquired hemophilia A (AHA) due to anti‐SARS‐CoV‐2 vaccination: A systematic review

Cited by 4 publications

References 37 publications

Severe acquired hemophilia A associated with COVID-19 vaccination: A case report and literature review

Severe acquired hemophilia A associated with COVID-19 vaccination: A case report and literature review

Haemophilia in the era of novel therapies: Where do inhibitors feature in the new landscape?

Acquired hemophilia A as a disease of the elderly: A comprehensive review of epidemiology, pathogenesis, and novel therapy

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