Acquired Hemophilia A: Current Guidance and Experience from Clinical Practice

Pishko, Allyson M.; Doshi, Bhavya S

doi:10.2147/jbm.s284804

Cited by 22 publications

(61 citation statements)

References 65 publications

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“…Although more than 50% of the cases are idiopathic ( Table 1 ), AHA has been associated with postpartum, drugs and underlying diseases like autoimmune disorders such as rheumatoid arthritis or systemic lupus erythematosus, malignancies and infectious diseases. 14–18 Medications reported to be associated with AHAs include penicillin, sulfonamides, phenytoin, interferons and fludarabine. 14 …”

Section: Epidemiologymentioning

confidence: 99%

“… 14–18 Medications reported to be associated with AHAs include penicillin, sulfonamides, phenytoin, interferons and fludarabine. 14 …”

Section: Epidemiologymentioning

confidence: 99%

“…The etiopathogenesis of drug-associated AHA is not well understood. 14 , 43 They mostly occur after hypersensitivity reactions and, despite generally high inhibitor titers, they remit after a short period after drug withdrawal with a remission rate close to 80%.…”

Section: Epidemiologymentioning

confidence: 99%

See 2 more Smart Citations

Acquired Haemophilia A: A Review of What We Know

Mingot‐Castellano¹,

Rodríguez-Martorell²,

Nuñez-Vázquez³

et al. 2022

JBM

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Autoantibodies against plasma coagulation factors could be developed by some individuals inducing severe and sometimes fatal bleedings. This clinical entity is called acquired haemophilia. It should be suspected in subjects with acute abnormal bleedings, without personal or familiar history of congenital bleeding disorders with an unexplained prolonged aPTT. It is rare disease, although its incidence may be underestimated due to the low knowledge about it by many specialists, the frequent use of anticoagulant or antiplatelet therapies in the affected population that can mask the diagnosis and, sometimes, a so withering effect that avoid its confirmation. Mortality ranges between 9% and 33% depending on the series in the first 2 months after diagnosis. This mortality is attributed in up to 40% of the cases to infections in the context of immunosuppressive treatments used to eliminate the inhibitor. Factor VIII levels below 1% and high inhibitor titers are conditions of worse response rates. Advanced age, patient’s ECOG, and underlying conditions are key prognostic factors for response to treatment and patient survival. To reduce morbidity and mortality in these patients, it is important to have clinical knowledge and access to guidelines to achieve an early diagnosis and to optimize the haemostatic and immunosuppressive treatment. This review aims to contribute to the dissemination of basic concepts on the epidemiology etiopathogenesis, diagnosis, treatment and management of these patients, as well as risk factors to get remission and the longest overall survival to allow individualized care. Especial awareness will be proposed in patients with some underlying conditions like cancer, autoimmune diseases, children, pregnancy or drugs.

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Section: Epidemiologymentioning

confidence: 99%

“… 14–18 Medications reported to be associated with AHAs include penicillin, sulfonamides, phenytoin, interferons and fludarabine. 14 …”

Section: Epidemiologymentioning

confidence: 99%

See 1 more Smart Citation

Acquired Haemophilia A: A Review of What We Know

Mingot‐Castellano¹,

Rodríguez-Martorell²,

Nuñez-Vázquez³

et al. 2022

JBM

View full text Add to dashboard Cite

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“…The bleeding pattern of AHA is rather different from that of congenital hemophilia A. Indeed, most patients with FVIII autoantibodies develops hemorrhages into the skin, muscles or soft tissues, and mucous membranes (e.g., epistaxis, gastrointestinal and urologic bleeds, retroperitoneal hematomas, postpartum bleeding), whereas hemarthrosis, a typical feature of congenital factor VIII deficiency, are uncommon [ 2 , 3 , 4 , 5 ]. The hemorrhages are often serious or life threatening as the disease may dramatically manifest with excessive bleeding following trauma or surgery or by cerebral hemorrhage.…”

Section: Introductionmentioning

confidence: 99%

A Diagnostic of Acquired Hemophilia Following PD1/PDL1 Inhibitors in Advanced Melanoma: The Experience of Two Patients and a Literature Review

et al. 2022

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Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the development of specific autoantibodies against factor VIII (FVIII). Immunotherapy is a recent therapeutic option that targets the patient’s self-tolerance against tumor cells. Because therapeutic effects of the immune checkpoint inhibitors (ICIs) are mediated by enhancing the immune response to restore antitumor immunity, autoimmune-related adverse effects can be seen in up to 80% of patients during treatment and after treatment. A rare hematologic ICIs-related adverse event is AHA. Hereafter we report two cases of AHA developed during anti-PD-1 immunotherapy for advanced melanoma: one secondary to treatment with nivolumab and one secondary to pembrolizumab. Both patients were treated with activated FVII (Novoseven®, Novo Nordisk, Bagsværd, Denmark) as hemostatic treatment combined with the eradication of antibodies anti-FVIII obtained with rituximab. In the last few years these drugs have significantly improved the therapeutic armamentarium for the management of AHA. Indeed, while FVIIa has proven to be an effective and safe tool for the treatment of acute bleeding related to FVIII autoantibodies, rituximab is a promising alternative for the autoantibodies’ elimination and the restoration of normal hemostasis. Our finding supports the use of this combination even in AHA secondary to ICIs treatment.

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“…Acquired Hemophilia A (AHA), also known as Acquired Factor VIII deficiency, is a bleeding disorder caused by autoantibody generation against coagulation Factor VIII (FVIII), leading to inhibition of its pro-coagulative effects and causing severe bleeding [ 1 , 2 ]. AHA is a rare disorder, having an incidence rate of 1.5 in 1 million per annum [ 3 ].…”

mentioning

confidence: 99%