Acquired Hemophilia A: The Bleeding Disorder That Sneaks Up on You

Published: Aug 23, 2024

Acquired hemophilia A is a rare but potentially life-threatening bleeding disorder that can suddenly develop in adults. Unlike inherited hemophilia, it occurs when the body's immune system mistakenly attacks a crucial blood clotting protein.
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Symptoms and Diagnosis

The hallmark of acquired hemophilia A is unexpected, severe bleeding, often appearing as large bruises or internal bleeding. Diagnosis can be tricky, as many doctors aren't familiar with this rare condition. Key tests include blood clotting studies and specific factor assays. A prolonged activated partial thromboplastin time (aPTT) that doesn't correct in mixing studies is a red flag.

Causes and Risk Factors

In about half of cases, no underlying cause is found. However, acquired hemophilia A can be associated with autoimmune disorders, cancer, pregnancy, or certain medications. The condition becomes more common with age, with the highest rates seen in people over 60. Postpartum women are another group at increased risk.
A rare bleeding disorder caused by the immune system attacking a crucial blood clotting protein, leading to severe and unexpected bleeding in adults.

Treatment Approaches

Treatment has two main goals: stop acute bleeding and eliminate the inhibitor causing the problem. For bleeding control, bypassing agents like recombinant factor VIIa or activated prothrombin complex concentrates are often used. To eliminate the inhibitor, immunosuppressive drugs such as corticosteroids and cyclophosphamide are typically prescribed. In some cases, rituximab may be added to the regimen.

Long-term Outlook

With proper treatment, many patients achieve remission. However, close monitoring is crucial as relapses can occur. The mortality rate has improved over time but remains significant, emphasizing the importance of prompt diagnosis and treatment. Patients who survive the initial bleeding episode generally have a good long-term prognosis.

Frequently Asked Questions

No, unlike classic hemophilia, it's not inherited.

It affects about 1-2 people per million annually.

Many patients achieve remission with treatment.

No, but it's most common in people over 60.

Bleeding control can be rapid, but eliminating inhibitors may take weeks.

Key Takeaways

Acquired hemophilia A is a rare but serious condition that requires prompt diagnosis and specialized treatment to prevent life-threatening complications.
If you're experiencing unexplained bruising or bleeding, don't hesitate to discuss your symptoms with Doctronic for personalized guidance.

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References

  1. Tiede A, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica. 2020;105(7):1791-1801.
  2. Knoebl P, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631.
  3. Collins PW, et al. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood. 2012;120(1):39-46.

This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic. Always discuss health information with your healthcare provider.

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