• Immune thrombocytopenia (ITP) and hemophilia are rare blood disorders that interfere with the body’s natural ability to stop bleeding.
• Although ITP and hemophilia share common symptoms, including excessive bleeding and bruising, they have distinct underlying causes.
• Hemophilia is a lifelong condition, but about 80 percent of pediatric ITP cases resolve on their own without treatment. Both diseases have various treatment options.

Blood clotting, the body’s natural process of stopping a damaged blood vessel from bleeding, is essential to your overall health. Health conditions known as blood disorders or bleeding disorders interfere with the body’s blood-clotting functions. Two such conditions, immune thrombocytopenia and hemophilia, share some commonalities, including causing bruising and excessive bleeding — but they also have some key differences, including their underlying causes and treatments.

In this article, we’ll explore the similarities and differences between ITP and hemophilia. Understanding their respective causes, symptoms, and treatment can help in managing each condition.

Blood Clotting: A Primer

Because ITP and hemophilia both impair blood clotting, it’s helpful to understand what blood clotting is and how it works.

When a blood vessel becomes ruptured — perhaps due to a cut, puncture wound, or blunt force trauma — blood begins to leak from the site. Bleeding may be visible through an open wound on your skin, or it may happen internally and you wouldn’t be able to see it right away.

Normally, a person’s natural blood-clotting processes will kick in to stop the bleeding. Blood clotting, also called coagulation, involves blood cells called platelets (or thrombocytes) clustering together at the damaged blood vessel to plug the hole, thereby stopping the bleeding. Cleveland Clinic notes that platelets act like a natural bandage for your body.

From there, small molecules called clotting factors cause strands of fibrin — a strong, flexible protein your body produces — to form a seal inside the wound. In the human body, there are various clotting factors (also known as coagulation factors), each labeled with a Roman numeral, such as clotting factor I, II, V, XII, and so on. With time, the wound in the blood vessel heals, and within a few days, the blood clot will dissolve. Overall, the platelets seal a wound by forming a temporary, loose “platelet plug,” while coagulation factors serve to strengthen and stabilize that clot.

ITP vs. Hemophilia: An Overview

ITP and hemophilia both interfere with the body’s blood-clotting process, but in different ways.

Notably, research in the form of a few case reports suggests that it’s possible, but very rare, for a person to have both ITP and hemophilia at the same time.

What Is ITP?

Immune thrombocytopenia also goes by the name immune thrombocytopenic purpura. It’s an autoimmune condition in which a person’s immune system creates antibodies that mistakenly seek and destroy their platelets. This process results in a low platelet count, which means the body can’t properly plug holes in damaged blood vessels.

Researchers aren’t sure what causes ITP. Like other autoimmune disorders, ITP can run in families, but researchers aren’t sure how often that happens. A person is at a higher risk of developing ITP if a parent or sibling has it.

Infections such as HIV or hepatitis can cause ITP in adults. Children most often develop the condition after catching a virus, such as the mumps or the flu.

ITP affects people of all races and ethnicities and of any age and sex, though it’s most common among males and females over 60. Around 9.5 people out of 100,000 in the U.S. have ITP, and researchers estimate about 200,000 people worldwide have the condition.

What Is Hemophilia?

Whereas ITP is an autoimmune condition that affects platelet count, hemophilia is a genetic bleeding disorder, meaning it’s caused fully or partially by a gene mutation. A person with hemophilia either lacks certain blood factor proteins involved in clotting or their blood factor proteins may have abnormalities. Without the proper amount of healthy clotting factors, the body can’t repair injured blood vessels to stop bleeding.

There are several types of hemophilia. Hemophilia A (known as classic hemophilia) and hemophilia B (also called Christmas disease) are the two most common. The main difference is that hemophilia A affects clotting factor VIII and hemophilia B affects clotting factor IX.

Usually, hemophilia is congenital, meaning it’s been passed down from a parent at birth. In most cases, the faulty gene is passed via the X chromosome from mother to son. About 20 percent of cases of hemophilia are acquired hemophilia, meaning it wasn’t passed down through genes. Acquired hemophilia is associated with pregnancy, cancer, multiple sclerosis, and other autoimmune conditions.

Like ITP, hemophilia affects people of all racial and ethnic groups equally. It’s much more common in males than females: Hemophilia A is diagnosed in as many as 1 in 5,000 male births worldwide, according to RareDiseaseAdvisor. Hemophilia A is approximately four times as common as hemophilia B.

ITP vs. Hemophilia: Symptoms

As bleeding disorders that affect clotting, ITP and hemophilia share several signs and symptoms, including:

• Unexplained or excessive bleeding — This may include spontaneous nosebleeds or bloody gums during a simple dental procedure.
• Easy bruising
• Heavy menstrual periods
• Hard-to-stop bleeding
• Blood in the urine or stool
• Bleeding into the joints and muscles
• Bleeding in the brain — This is a very rare but potentially life-threatening sign of both conditions, characterized by symptoms including persistent headaches, double vision, or extreme sleepiness.

As noted above, bruising is a common symptom in both ITP and hemophilia, but the bruises tend to have different characteristics. Petechiae, which resembles tiny reddish-purple spots or a rash, is more common in ITP and usually doesn’t occur in hemophilia.

This symptom is caused by capillary blood leakage. People with ITP also sometimes develop purpura, larger purplish bruises.

In ITP, bruising can appear at the joints of elbows and knees, caused by “leaked blood.” A bruise forms when small blood vessels beneath the skin break, allowing blood to escape and pool under the skin’s surface. The pooled blood appears as a purple or reddish mark on light skin. If you have a darker skin tone, you may notice purple, dark brown, or black bruising.

Learn more about what ITP bruises look like.

People with hemophilia are more likely to develop many large, deep bruises following minor injuries. They’re a sign of bleeding under the skin.

In moderate and severe hemophilia, internal bleeding can also affect the joints, frequently affecting the shoulders, hips, and knees. This condition can lead to bruising, pain, aching, or swelling in the affected areas, and they may also feel warm to the touch.

ITP vs. Hemophilia: Diagnosis

ITP and hemophilia share similar symptoms with one another — and with other bleeding disorders, such as von Willebrand disease. Therefore, a health care provider may need to conduct a few tests to figure out what’s causing a person’s symptoms.

In diagnosing both conditions, a doctor will review your medical history and ask about symptoms and family history of bleeding disorders. During a physical exam, they’ll check for symptoms like pain, bleeding on or beneath the skin, and swelling associated with joint bleeds.

A complete blood count (CBC) can help determine whether clotting problems are caused by low levels of platelets — a telltale sign of ITP that could rule out hemophilia.

More tests may be necessary for diagnosing hemophilia than ITP. These tests could include a clotting factor assay, which establishes how much of each clotting factor a person has in their blood, and a partial thromboplastin time test, which measures how quickly a person’s blood clots.

ITP vs. Hemophilia: Treatments

Given that ITP and hemophilia have different underlying causes, they have significantly different treatments. ITP treatment tends to focus on increasing platelet counts, whereas hemophilia treatments work to compensate for clotting-factor issues.

Treatments for ITP

People with mild ITP may not need treatment at all, beyond regular platelet checks. Children living with ITP often improve without any kind of treatment. But for many adults, ITP worsens over time and most will eventually need treatment.

Learn about the five signs of untreated ITP and ways to manage it.

If a person living with ITP does require treatment, a doctor may prescribe medications such as:

• Steroids — Help to reduce inflammation
• Immunoglobulin — Antibodies
• Thrombopoietin receptor agonists — Help bone marrow produce more platelets
• Immunosuppressives — Reduce the immune system response that damages platelets

Side effects vary from medication to medication and person to person, so make sure to ask about them when starting a new prescription.

In more severe cases, a doctor may recommend a splenectomy (removal of the spleen). The spleen produces the antibodies that attack platelets in ITP. It also destroys antibody-coated platelets so removing it may help — though it can put you at greater risk of infection. Your doctor may also recommend a platelet transfusion, which is when platelets from a donor are injected into your bloodstream.

Learn more about 10 common treatments for ITP and four new advances in chronic ITP treatment.

Treatments for Hemophilia

Whereas ITP treatment often focuses on increasing platelet counts and calming down the immune system, treatment for hemophilia focuses on replacing clotting factors. Replacement clotting factors can be made from donated blood or produced in a lab, called recombinant clotting factors.

In mild cases of hemophilia, a hematologist also may prescribe desmopressin, a hormone that can coax more clotting factor out of your body. Antifibrinolytics, also known also as clot-preserving medications, can help prevent clots from breaking down.

Find Support

On myITPcenter, the site for people with immune thrombocytopenia and their loved ones, people come together to gain a new understanding of ITP and share their stories with others who understand life with the condition.

What questions do you have about ITP or hemophilia? What tips can you share with others living with a bleeding disorder? Share your questions and advice in a comment below.