Acquired hemophilia A following COVID-19 vaccination – The importance of prompt diagnosis: A case report

Duminuco, Andrea; Calagna, Marianna; Markovic, Uroš; Esposito, Benedetta; Grasso, Stephanie; Riccobene, Carla; Raimondo, Francesco Di; Giuffrida, Gaetano

doi:10.1016/j.transci.2022.103577

Cited by 7 publications

(5 citation statements)

References 18 publications

(17 reference statements)

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“…Evidence to date has shown that the effectiveness of the two vaccines in preventing infection by the SARS-CoV-2 virus ranges from 94-95% [30]. In the setting of hematological patients, we previously studied and reported the risk of vaccine exacerbating an underline coagulative disorder (i.e., hemophilia or thrombotic thrombocytopenic purpura [31,32]). It's known the role of the disrupted immune system and the previous or concomitant therapy in not allowing an adequate antibody response to administering the vaccine [33][34][35][36][37].…”

Section: Discussionmentioning

confidence: 99%

Breakthrough infection after SARS-CoV-2 vaccines in patients with hemoglobinopathies and blood-transfusion management: a single Center experience

Duminuco,

Bulla,

Rosso

et al. 2023

Preprint

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Purpose Immune system impairment is frequently reported in patients affected by hemoglobinopathies due to various mechanisms, including iron accumulation, antigenic stimulation due to numerous transfusions, chronic hemolysis, and a hyperinflammatory state. The antigenic immune response after a vaccine could be ineffective. Methods We evaluated the anti-spike IgG production after 2 doses of vaccine for SARS-CoV-2 in patients affected by hemoglobinopathies, reporting the risk of breakthrough infections, monitoring the outcome and the risk of severe disease or complications related to the basal hematological disease. Results All 114 enrolled patients developed adequate antibody production, with a median value of serum anti-S IgG of 2184.4 BAU/mL. The amount of antibody was unrelated to any other clinical characteristics evaluated, including transfusion dependence, age, gender, disease type, ferritin, blood count, spleen status, and therapy with hydroxyurea or iron chelators (p > 0.05). Moreover, 47 (41.2%) patients developed breakthrough SARS-CoV-2 infection during the follow-up, all with a mildly symptomatic course, without requiring hospitalization or experiencing a significative drop in hemoglobin values, allowing for a slight delay in their transfusion regimen. Conclusion Vaccination has been an effective and safe tool in this category of patients, preventing severe complications. Watchful waiting in the transfusion strategy can be safely ensured, guaranteeing better management of transfusion components.

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Section: Discussionmentioning

confidence: 99%

Breakthrough infection after SARS-CoV-2 vaccines in patients with hemoglobinopathies and blood-transfusion management: a single Center experience

Duminuco,

Bulla,

Rosso

et al. 2023

Preprint

Self Cite

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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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“…Interstingly, the elevation of FVIII has been also reported in long-COVID in association with thrombo-inflammatory manifestations and vascular dysfunction [ 84 , 85 ]. Also, very few cases of acquired hemophilia A, a bleeding condition caused by the development of auto-antibodies against FVIII, has been observed after SARS-CoV-2 S mRNA-based vaccination, perhaps as the result of a cross-reaction between antibodies against S protein and endogenous FVIII [ 86 ]. More commonly, antiphospholipid antibodies (aPL) derived from B1-cells induced up-regulation of C-reactive protein and D-dimer, and were related to fatal outcomes in COVID-19 patients.…”

Section: Disbalanced Endothelial-derived Hemostatic Regulators In Cov...mentioning

confidence: 99%

Mechanisms of endothelial activation, hypercoagulation and thrombosis in COVID-19: a link with diabetes mellitus

Valencia,

Lumpuy-Castillo,

Magalhaes

et al. 2024

Cardiovasc Diabetol

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Early since the onset of the COVID-19 pandemic, the medical and scientific community were aware of extra respiratory actions of SARS-CoV-2 infection. Endothelitis, hypercoagulation, and hypofibrinolysis were identified in COVID-19 patients as subsequent responses of endothelial dysfunction. Activation of the endothelial barrier may increase the severity of the disease and contribute to long-COVID syndrome and post-COVID sequelae. Besides, it may cause alterations in primary, secondary, and tertiary hemostasis. Importantly, these responses have been highly decisive in the evolution of infected patients also diagnosed with diabetes mellitus (DM), who showed previous endothelial dysfunction. In this review, we provide an overview of the potential triggers of endothelial activation related to COVID-19 and COVID-19 under diabetic milieu. Several mechanisms are induced by both the viral particle itself and by the subsequent immune-defensive response (i.e., NF-κB/NLRP3 inflammasome pathway, vasoactive peptides, cytokine storm, NETosis, activation of the complement system). Alterations in coagulation mediators such as factor VIII, fibrin, tissue factor, the von Willebrand factor: ADAMST-13 ratio, and the kallikrein-kinin or plasminogen-plasmin systems have been reported. Moreover, an imbalance of thrombotic and thrombolytic (tPA, PAI-I, fibrinogen) factors favors hypercoagulation and hypofibrinolysis. In the context of DM, these mechanisms can be exacerbated leading to higher loss of hemostasis. However, a series of therapeutic strategies targeting the activated endothelium such as specific antibodies or inhibitors against thrombin, key cytokines, factor X, complement system, the kallikrein-kinin system or NETosis, might represent new opportunities to address this hypercoagulable state present in COVID-19 and DM. Antidiabetics may also ameliorate endothelial dysfunction, inflammation, and platelet aggregation. By improving the microvascular pathology in COVID-19 and post-COVID subjects, the associated comorbidities and the risk of mortality could be reduced.

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Acquired hemophilia A following COVID-19 vaccination – The importance of prompt diagnosis: A case report

Cited by 7 publications

References 18 publications

Breakthrough infection after SARS-CoV-2 vaccines in patients with hemoglobinopathies and blood-transfusion management: a single Center experience

Breakthrough infection after SARS-CoV-2 vaccines in patients with hemoglobinopathies and blood-transfusion management: a single Center experience

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

Mechanisms of endothelial activation, hypercoagulation and thrombosis in COVID-19: a link with diabetes mellitus

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