Acquired hemophilia A (AHA): underreported, underdiagnosed, undertreated medical condition

Demerdash, Doaa El; Ayad, Alia; Tawfik, Nehad M.

doi:10.1186/s43162-021-00074-9

Cited by 4 publications

(13 citation statements)

References 25 publications

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“… 2 However, our patients were younger compared to the reported peak age of more than 60 years of age. 11 Female patients presenting with AHA are generally younger and pregnant. Our patients were female, with a background history of HIV, spontaneously bleeding, had prolonged APTT’s and no correction on the APTT after 50:50 mixing studies.…”

Section: Discussionmentioning

confidence: 99%

The diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa

Pillay,

Rapiti

2023

J Prim Care Community Health

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Introduction: In acquired hemophilia A (AHA), the body produces auto-antibodies against Factor VIII. Although AHA is rare, with an incidence of 1.5 patients/1 million population/year, there is a strong association with human immunodeficiency virus (HIV) infection. The accurate interpretation of screening coagulation tests is critical to identify patients with AHA, as the mortality rate secondary to bleeding is high. Methods: This was a retrospective case series which included all newly diagnosed AHA patients that were referred to Hemophilia care unit at King Edward VIII Hospital, Durban, South Africa from January 2011 to December 2021. The clinical presentation and laboratory results were documented. Results: Five patients were included in this case series. All patients were females aged between 28 and 64 years of age and they were HIV seropositive. They presented with spontaneous cutaneous and intramuscular bleeding. Four patients were virologically suppressed on anti-retroviral therapy, and no patient had a family history of congenital bleeding diathesis. Laboratory investigations confirmed AHA with high Factor VIII inhibitor titers, which ranged from 41 to 900 Bethesda Units (BU). All patients were managed with bypassing agents and oral corticosteroids. The monitoring of patients after the initiation of treatment was difficult as they all defaulted treatment. Conclusion: In view of the prevalence of HIV in sub-Saharan Africa, there is a possibility that AHA is under-diagnosed in our setting. The clinician and the laboratory have a combined critical role in identifying patients with AHA as the investigation of a prolonged APTT is mandatory. There are challenges in managing AHA patients in a resource-constrained setting.

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Section: Discussionmentioning

confidence: 99%

The diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa

Pillay,

Rapiti

2023

J Prim Care Community Health

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“…We present the case of a patient with postpartum AHA, two months after her second pregnancy, who progressed favorably after treatment with activated recombinant factor VII. The prevalence rate of this condition is 8.4% of all cases of acquired hemophilia 4 . Cases of postpartum AHA after the second pregnancy are rare, since 80% of the cases occur after the first pregnancy 4 .…”

Section: Discussionmentioning

confidence: 99%

“…The age distribution of AHA is usually biphasic, with a first peak between 20 and 30 years of age, mainly in young women during pregnancy and postpartum, and a second peak in those over 60 years of age, with no gender differences 3 . The mortality rate is 41% if patients do not receive treatment within the first week 4 .…”

Section: Introductionmentioning

confidence: 99%

“…The cause of AHA is unknown in 50% of the cases; of the remaining cases, 7-21% are related to the postpartum period 5 . The mortality rate of postpartum-acquired hemophilia A ranges from 12 to 22% and it may occur in pregnant women who present bleeding without apparent cause and who have no history of bleeding disorders 4 . It usually occurs after the first pregnancy in 80% of cases, and generally one to four months after delivery 6 , 7 .…”

Section: Introductionmentioning

confidence: 99%

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Hemofilia A adquirida en el posparto: reporte de caso

Contreras-Pizarro,

Chumpitaz-Anchiraico,

Ticona Sanjínez

et al. 2023

Rev Peru Med Exp Salud Publica

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La hemofilia A adquirida es un trastorno hemorrágico poco frecuente a nivel mundial, y se caracteriza por la presencia de autoanticuerpos inhibidores dirigidos hacia un factor de la coagulación, en la mayoría de ocasiones el factor VIII. Las etiologías son variadas, entre las que se encuentra el posparto. Se presenta el caso de una paciente de 34 años con dolor lumbar, hematuria y hematoma en región glútea derecha, sin antecedentes previos de sangrado. Por extensión de las manifestaciones hemorrágicas es transferida al servicio de emergencia. Los exámenes auxiliares de perfil de coagulación, prueba de mezclas y medición de los títulos de inhibidores del factor VIII permitieron confirmar el diagnóstico. El caso resalta la importancia de considerar esta patología en una paciente puérpera con persistencia de sangrado por herida operatoria, hematoma extenso y sin historia de sangrado previo.

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“…AH is characterized by factor VIII neutralizing autoantibodies that affect both men and women with a presumed incidence of 1 to 1.5 per million individuals per year [2,4]. In general, it affects the elderly aged 64 to 78 with many comorbidities and on various medications, including anticoagulants and antiplatelet agents, as in the first case described, which can influence the clinical picture and require an individualized therapeutic approach [5].…”

Section: Discussionmentioning

confidence: 99%

"Acquired Hemophilia: Rare or Underdiagnosed Pathology. Report of Two Cases"

Côrtes

2022

BJSTR

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Background: Acquired hemophilia (AH) is caused by the presence of factor VIII inhibitor and can be associated with various etiologies: hematological neoplasms or solid tumors, drugs, autoimmune diseases, pregnancy, etc. Through a review of medical records, two cases of the disease are described in male patients older than 70, diagnosed over three years: from 2019 to 2021 in our hospital. Case Presentation:The first case refers to a 72-year-old patient with hematuria, who developed ecchymosis and subcutaneous hematomas associated with prolonged activated thromboplastin time (aPTT), reduced blood levels of factor VIII and factor VIII inhibitor. The second case refers to an 89-year-old patient with major anemia, ecchymosis and hematomas all over the body and the same hematological profile as the first case. Both cases responded to the immunosuppressive therapy with complete remission of clinical and laboratory abnormalities. We report the clinical manifestations on admission, the laboratory abnormalities, and the therapeutic regimen in each case. Acquired hemophilia (AH) is a disease little known by the medical community. Its main signs are hemorrhagic phenomena associated or not with anemia, prolonged aPTT, drop in serum factor VIII levels and presence of factor VIII inhibitor. Conclusion:High-level clinical suspicion is imperative to diagnose the disease and start the proper therapy. of systemic arterial hypertension (SAH), diabetes mellitus (DM),

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Acquired hemophilia A (AHA): underreported, underdiagnosed, undertreated medical condition

Cited by 4 publications

References 25 publications

The diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa

The diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa

Hemofilia A adquirida en el posparto: reporte de caso

"Acquired Hemophilia: Rare or Underdiagnosed Pathology. Report of Two Cases"

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