Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder

Yousphi, Adeel S; Bakhtiar, Ayesha; Cheema, Muhammad Arslan; Nasim, Syed; Ullah, Waqas

doi:10.7759/cureus.5442

Cited by 16 publications

(19 citation statements)

References 14 publications

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“…In most cases, autoantibodies are formed against factor VIII, which is known as acquired haemophilia A (2). But in our case, inhibitors were present against both factor VIII and IX, which has the incidence as 1 per million per year, with many cases being undiagnosed (5).…”

Section: Discussionmentioning

confidence: 48%

“…Acquired haemophilia is a haematological disorder caused by the formation of antibodies commonly against factor VIII and rarely against factor IX and other clotting factors. It is a rare bleeding disorder common in the elderly, where the commonest presentation is spontaneous bleeding (5). In most cases, autoantibodies are formed against factor VIII, which is known as acquired haemophilia A (2).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Idiopathic acquired haemophilia with inhibitors to factor VIII and IX in an elderly female: a case report

Rupasinghe¹,

Kularatne²,

Dissanayaka³

2020

Anuradhapura Med. J.

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Section: Discussionmentioning

confidence: 48%

Section: Discussionmentioning

confidence: 99%

Idiopathic acquired haemophilia with inhibitors to factor VIII and IX in an elderly female: a case report

Rupasinghe¹,

Kularatne²,

Dissanayaka³

2020

Anuradhapura Med. J.

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“…Although it has been shown that the bleeding symptoms in AHA are usually severe, physicians should also be aware that mild bleeding can also occur in AHA [9,16,41]. A thorough laboratory examination must be performed to [2,6,12,16].…”

Section: Lessons Learnedmentioning

confidence: 99%

Diagnosis and Management of Acquired Hemophilia A: Case Reports and a Literature Review

Rinaldi

Prasetyawaty

Fazlines

et al. 2021

Case Reports in Medicine

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Background. Acquired hemophilia A (AHA) is a potentially life-threatening autoimmune hemostatic disorder where autoantibodies that disrupt the functions of factor VIII (FVIII) are present in the circulation. The early diagnosis of AHA is difficult since the symptoms of AHA differ from those of congenital hemophilia A. Furthermore, the management of AHA is also more complex due to the presence of autoantibodies against FVIII (FVIII inhibitors). Here, we present three case reports and conduct a literature review of AHA with the aim to increase awareness and knowledge regarding the diagnosis and treatment of AHA. Case Presentations. We present three patients diagnosed with AHA in these case reports. The first patient was a young female, while the second and third patients were middle-aged and elderly males, respectively. All patients presented with a chief complaint of bruises without hemarthrosis and a history of bleeding. Laboratory examinations of the patients revealed isolated prolonged aPTT, normal PT, and the presence of autoantibodies against factor VIII, which are characteristics of AHA. Patients were then treated with corticosteroids to reduce the titer level of autoantibodies and received factor VIII transfusion to stop bleeding. Conclusion. AHA can be suspected in patients presenting with symptoms of bruises without hemarthrosis and without the history of bleeding. Isolated aPTT elevation with normal PT should raise high suspicion of AHA. The presence of FVIII inhibitors can help to confirm the diagnosis of AHA. Treatment consists of factor VIII transfusion and corticosteroid therapy. Bypassing agents are recommended as an alternative to FVIII transfusion.

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“…Most patients with F8 autoantibodies have hemorrhages in the skin, muscles, or soft tissues and mucous membranes, whereas hemarthrosis, a typical feature of congenital F8 deficiency, is uncommon. This disease develops during a person's lifetime, usually in special conditions, such as pregnancy, after major surgery or very often linked to underlying malignancy, and it is described as an autoimmune disease (40,41). In acquired hemophilia, the newly developed antibodies are different from the alloantibodies developed as a response to rF8 treatment in congenital HA (39) (Figure 1).…”

Section: Aha-a Recently Described Ha With a Non-mutational Backgroundmentioning

confidence: 99%

The Possible Non-Mutational Causes of FVIII Deficiency: Non-Coding RNAs and Acquired Hemophilia A

et al. 2021

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Hemophilia type A (HA) is the most common type of blood coagulation disorder. While the vast majority of cases are inherited and caused by mutations in the F8 gene, recent data raises new questions regarding the non-heritability of this disease, as well as how other molecular mechanisms might lead to the development of HA or increase the severity of the disease. Some data suggest that miRNAs may affect the severity of HA, but for some patients, miRNA-based interference might cause HA, in the absence of an F8 mutation. A mechanism in HA installation that is also worth investigating and which could be identified in the future is the epigenetic silencing of the F8 gene that might be only temporarily. Acquired HA is increasingly reported and as more cases are identified, the description of the disease might become challenging, as cases without FVIII autoantibodies might be identified.

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Acquired Hemophilia A: A Rare but Potentially Fatal Bleeding Disorder

Cited by 16 publications

References 14 publications

Idiopathic acquired haemophilia with inhibitors to factor VIII and IX in an elderly female: a case report

Idiopathic acquired haemophilia with inhibitors to factor VIII and IX in an elderly female: a case report

Diagnosis and Management of Acquired Hemophilia A: Case Reports and a Literature Review

The Possible Non-Mutational Causes of FVIII Deficiency: Non-Coding RNAs and Acquired Hemophilia A

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