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Acquired Hemophilia A

Acquired Hemophilia A

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Diseases & Conditions
Blood Disorders
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Contributed byKrish Tangella MD, MBANov 08, 2019

What are the other Names for this Condition? (Also known as/Synonyms)

  • Acquired Factor 8 Deficiency Disorder
  • Acquired Factor VIII Deficiency Disorder

What is Acquired Hemophilia A? (Definition/Background Information)

  • Acquired Hemophilia A is a bleeding disorder that interferes with the body's blood clotting process. Although the condition can affect people of all ages, it generally occurs in older people (the median age of diagnosis is between 60 and 67 years)
  • Signs and symptoms include prolonged bleeding, frequent nosebleeds, bruising throughout the body, solid swellings of congealed blood (hematomas), hematuria, and gastrointestinal or urologic bleeding
  • Acquired Hemophilia A occurs when the body's immune system attacks and disables a certain protein that helps the blood clot (called coagulation factor VIII)
  • About half of the cases are associated with other conditions, such as pregnancy, autoimmune disease, cancer, skin diseases, or allergic reactions to medications
  • Treatment is aimed at controlling bleeding episodes and addressing the underlying cause of the condition

(Source: Acquired Hemophilia A; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Acquired Hemophilia A? (Age and Sex Distribution)

  • Acquired Hemophilia A is a rare bleeding condition typically seen in older adults (diagnosis median age is about 60-67 years). But, it may affect individuals of a wider age group
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Acquired Hemophilia A? (Predisposing Factors)

The risk factors for Acquired Hemophilia A may include the following set of associated conditions:

  • Autoimmune disorder (such as lupus, rheumatoid arthritis, etc.)
  • Uncontrolled diabetes
  • Pregnancy (generally in the post-partum period)
  • Cancer
  • Certain medications (such as penicillin, interferon)
  • Skin diseases

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acquired Hemophilia A? (Etiology)

  • Acquired Hemophilia A is caused by antibodies generated by an individual’s body against clotting factor VIII, leading to a deficiency of this factor
  • The production of autoantibodies can be generated by pregnancy, autoimmune disorders, cancer, medication side effects, and other conditions

What are the Signs and Symptoms of Acquired Hemophilia A?

The signs and symptoms of Acquired Hemophilia A may include:

  • Prolonged bleeding
  • Frequent nosebleeds 
  • Bruising throughout the body
  • Solid swellings of congealed blood (hematomas)
  • Hematuria
  • Gastrointestinal or urologic bleeding

How is Acquired Hemophilia A Diagnosed?

Acquired Hemophilia A is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acquired Hemophilia A?

The complications of Acquired Hemophilia A may include:

  • Intra-cerebral hemorrhage - bleeding in the brain
  • Joint deformities, due to persistent bleeding into the joint space
  • Allergic reaction to infusion of blood products
  • Internal bleeding in various organs
  • Formation of hemophilic pseudotumor
  • Relapse of bleeding episodes following treatment

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Acquired Hemophilia A Treated?

The treatment for Acquired Hemophilia A is decided on a case-by-case basis, and may depend on the underlying condition causing Acquired Hemophilia A, and an individual’s tolerance to treatment. 

The following treatment methods may be employed:

  • Treatment of the underlying cause of Acquired Hemophilia A (if any)
  • Identifying triggers for bleeding and preventing bleeding episodes
  • Controlling episodes of bleeding: Use of bypassing agents, which decrease the need for factor VIII for the clotting process
  • Immune-suppression to remove autoantibodies, which may be accomplished by administering the following:
    • Corticosteroids
    • Cyclophosphamide

How can Acquired Hemophilia A be Prevented?

  • Acquired Hemophilia A may be prevented or its risk minimized, with the treatment of the underlying disease, or identifying and removing the trigger for the abnormal immune reaction
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired disorders
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Acquired Hemophilia A? (Outcomes/Resolutions)

  • The prognosis of Acquired Hemophilia A is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Individuals with mild conditions have better prognosis than those with severe symptoms and complications
  • Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Acquired Hemophilia A:

The following DoveMed website links are useful resources for additional information:

http://www.dovemed.com/diseases-conditions/hemophilia-a/

http://www.dovemed.com/diseases-conditions/rare-disorders/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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