Most hemophilia cases develop in people with gene mutations (changes) that affect clotting factor proteins. In more rare cases, a person can have acquired hemophilia. This bleeding disorder occurs when the immune system attacks clotting factors. As a result, your blood can’t properly make clots.

This article will discuss what acquired hemophilia is, who develops it, and how it’s diagnosed. We’ll also cover how doctors treat acquired hemophilia and how it’s different from other types. To learn more about acquired hemophilia, talk to your doctor.

What Is Hemophilia?

Hemophilia is a disorder that causes excessive bleeding because the blood doesn’t clot properly. People with hemophilia have low levels of clotting factors — specialized proteins that work together to form clots. Blood clots are made of sticky cell fragments called platelets, along with strands of fibrin protein.

There are three main types of hemophilia. Each affects a different clotting (coagulation) factor:

• Hemophilia A — Affects clotting factor VIII (8)
• Hemophilia B — Affects clotting factor IX (9)
• Hemophilia C — Affects clotting factor XI (11)

What Is Congenital Hemophilia?

Most cases of hemophilia are congenital, meaning they’re present at birth. Congenital hemophilia typically occurs when a person has mutations in their clotting factor genes.

Every person gets two copies of every gene — one from each parent. If a parent has hemophilia or carries a mutated gene, their child has a higher risk of developing the condition. Mutations in clotting factors VIII and IX are passed down on the X chromosome. Females typically have two X chromosomes, according to MedlinePlus, and males typically have one X and one Y chromosome.

Males who inherit an X chromosome with a faulty clotting factor gene will develop hemophilia, according to Cleveland Clinic. Females who inherit one faulty and one healthy X chromosome are typically “carriers.” Although carriers may not show symptoms, some might experience mild bleeding issues. Carriers can pass the faulty gene to their children.

What Is Acquired Hemophilia?

Acquired hemophilia is an autoimmune disorder. This means that the immune system mistakenly attacks the body’s healthy cells. Normally, your immune system protects you from foreign invaders. It creates specialized proteins called antibodies, which signal other immune cells to attack threats.

In acquired hemophilia, your immune system makes autoantibodies that target clotting factor proteins. This destroys the factors and affects how well your blood makes clots. Clotting factor VIII is the most common target.

Acquired hemophilia isn’t a genetic disorder. The term “acquired” indicates that the disease developed at some point after birth. People with acquired hemophilia don’t have any family history of the condition.

Who’s at Risk of Developing Acquired Hemophilia?

Research shows that acquired hemophilia tends to develop in the elderly. Around half of all cases occur in people with other autoimmune disorders and health conditions. Examples include:

• Inflammatory bowel disease (IBD), such as ulcerative colitis
• Sjögren’s syndrome
• Lupus
• Multiple sclerosis
• Rheumatoid arthritis
• Pregnancy, especially in individuals who have recently given birth
• Certain medications, including penicillin
• Infections like hepatitis B and hepatitis C
• Certain cancers, including cancers of the lungs, breast, colon and blood

The other 50 percent of acquired hemophilia cases have no known cause. Doctors and researchers are still unsure why the condition develops in some people.

What Are the Symptoms of Acquired Hemophilia?

People with acquired hemophilia can bleed very easily because their blood can’t form clots effectively. Sometimes, bleeding occurs spontaneously and may be severe. Left uncontrolled, these bleeding episodes can become life-threatening.

Symptoms of acquired hemophilia include:

• Easy bruising and bleeding
• Blood in your urine (pee)
• Bleeding in the muscles and soft tissues
• Easy bleeding during dental procedures or surgery
• Gastrointestinal bleeding
• Heavy menstrual periods and heavy bleeding after giving birth
• Bleeding in the brain, which is rare

Acquired hemophilia can also cause joint bleeding, but this is much less common compared to congenital hemophilia A.

How Is Acquired Hemophilia Diagnosed?

If your doctor thinks you may have acquired hemophilia, they’ll run a series of tests. You’re more likely to have acquired hemophilia if you’re an older adult or you’ve recently given birth.

Doctors usually start with laboratory tests that measure how well your blood clots. These tests look at different coagulation factors. You’ll need to give a small blood sample.

An activated partial thromboplastin time (APTT) test looks at factors VIII, IX, XI, and XII (12). A prothrombin time (PT) test looks at factors II (2), VII (7), and IX.

People with acquired hemophilia have a prolonged APTT test. This means that it takes longer for blood to make clots than normal. PT test results are usually normal.

Measuring Clotting Factor and Antibody Levels

Hemophilia develops when you have very low levels of clotting factors. According to the Centers for Disease Control and Prevention (CDC), hemophilia is classified as mild, moderate, or severe. The severity depends on your clotting factor levels.

Your doctor will order tests to measure how much of each clotting factor is in your blood. They’ll also measure your antibody titers (levels) to determine the severity of your acquired hemophilia.

How Is Acquired Hemophilia Treated?

After you’ve been diagnosed with acquired hemophilia, your doctor will create a treatment plan. Your plan will depend on how severe your hemophilia is and any other conditions you may have.

In some people, acquired hemophilia goes away on its own, a phenomenon known as spontaneous remission. This is more common in those with low autoantibody levels. Researchers don’t know why acquired hemophilia goes away on its own.

Treatments To Dampen the Immune System

Some acquired hemophilia treatments focus on stopping the immune system from making autoantibodies. They’re known as immunosuppressive treatments because they suppress or dampen your immune system. This helps protect your clotting factors from being destroyed.

Examples of these medications include:

• Corticosteroids (steroids) like prednisone
• Certain chemotherapy drugs like cyclophosphamide

Treating Bleeding Episodes

If you have severe bleeding episodes, you’ll need treatment to stop them. The U.S. Food and Drug Administration (FDA) has approved a few treatments.

The first recommended treatment option (first-line therapy) is a bypassing agent. One example is recombinant (lab-made) proteins like activated factor VII (rFVIIa). This is sold under the brand name NovoSeven RT. Recombinant factors don’t contain any blood or proteins from other humans.

Another treatment option is activated prothrombin complex concentrate (aPCC), such as Feiba. It’s an FDA-approved treatment for managing bleeding in acquired hemophilia.

It’s important to note that bypassing agents don’t work for everyone. Your doctor may need to try other treatments. For those experiencing acute bleeding, surgery may be necessary to control and stop it.

Talk to Your Doctor About Acquired Hemophilia

If you have any questions about acquired hemophilia or other bleeding disorders, talk to your doctor. They’ll manage your treatment plan and make recommendations to help prevent bleeding episodes. Your doctor can also recommend a hemophilia treatment center. These centers have health care teams trained to treat bleeding disorders.

Find Your Team

On MyHemophiliaTeam, the social network for people with hemophilia and their loved ones, more than 6,600 people from around the world come together to ask questions, offer support and advice, and connect with others who understand life with hemophilia.

Are you or a loved one living with acquired hemophilia? Do you have questions about this rare bleeding disorder? Share them below, or post a comment on your Activities page.