Acquired hemophilia is a rare condition that is a disease of the immune system. With this condition, the body’s immune system targets clotting factors in the blood, such as factor VIII. Acquired hemophilia can occur in people that have rheumatoid arthritis and can have serious consequences.
Hemophilia is a disorder that stops the blood from clotting properly. For most people with the condition, it is an inherited disease. Reduced levels of certain blood clotting factors can cause bleeding and trigger serious health problems in some severe cases. There are two types of hemophilia: A and B. Both can be congenital, which means a person inherits it, or they can acquire it, meaning it develops later in life.
Rheumatoid arthritis is an autoimmune disease that mainly affects the joints. The body’s immune defenses mistakenly attack the joints in the hand, wrist, or knee, causing inflammation and damage. Besides chronic joint pain, balance problems, and deformed bones, the condition can also affect organs such as the lungs, heart, and eyes.
A link between both conditions exists. Acquired hemophilia has associations with rheumatoid arthritis in about
Keep reading to learn more about the link between hemophilia and rheumatoid arthritis, including diagnosing and treating both conditions.
Acquired hemophilia is similar to congenital hemophilia in that it can cause bleeding. However, the acquired form of the disease is a rare immune system disorder that involves the immune system attacking healthy cells.
Unlike congenital hemophilia, acquired hemophilia does not pass down through generations of a family. Additionally, congenital hemophilia only affects males, whereas acquired hemophilia can affect people of any sex.
In acquired hemophilia, the body makes antibodies that attack proteins, known as clotting factors. With these proteins depleted, the blood does not clot as it should. An example of an affected clotting factor is factor VIII. When blood clots, it clumps together at the site of a wound like a plug.
People who have acquired hemophilia can experience a range of serious complications of the disease due to atypical and uncontrolled bleeding into the soft tissue, skin, and muscles. Sometimes this happens spontaneously or due to surgery or trauma.
Can rheumatoid arthritis cause hemophilia?
According to a recent case report, acquired hemophilia can have links to cancer, pregnancy, certain medications, or autoimmune conditions, including:
However, half of all cases of acquired hemophilia have no identifiable cause.
Both acquired hemophilia and rheumatoid arthritis are immune disorders, where the body attacks its own healthy cells.
Many people with a diagnosis of one autoimmune condition go on to develop other autoimmune disorders. Researchers working on a
Polyautoimmunity refers to the state in which a person has
Symptoms can occur when the blood is not clotting properly. People with acquired hemophilia might experience the following:
- subcutaneous bleeding, or bleeding under the skin
- hematoma, or pools of blood outside the blood vessels
- melena, or dark feces containing partially digested blood
- hematuria, or blood in the urine
- intracranial hemorrhage, a rare and possibly fatal symptom
Acquired hemophilia — secondary to rheumatoid arthritis
According to a 2022 case report, a 69-year-old female, who had lived with rheumatoid arthritis for 25 years, experienced a severe nosebleed and bleeding into her muscles and skin. When doctors performed tests, they found that she had factor VIII clotting factors below healthy levels in her blood.
An older case report from 2016 involved another 69-year-old woman who required hospitalization. Doctors diagnosed her with rheumatoid arthritis 6 years before. Four months before the hospital admission, she experienced:
- bleeding into the skin of her left leg one day after a minor injury
- blood in her urine
- bruising to her trunk, upper limbs, and face
Doctors can diagnose both conditions by assessing their symptoms and results from the laboratory.
Doctors may diagnose acquired hemophilia if a person is experiencing atypical bleeding that has only started recently. They use a variety of specialized blood tests that measure how long it takes the person’s blood to clot. Some tests might include:
- Routine coagulation tests: These tests measure the time it takes for a clot to form. They can include checking prothrombin time (PT) and activated partial thromboplastin time (aPTT). A person with acquired hemophilia may have isolated aPTT and typical PT.
- Other tests to rule out potential causes of aPTT elevation: This can include checking for inhibitors in the blood or if the person is on any medication that can affect the results
- aPTT mixing tests: This test can further confirm the diagnosis of acquired hemophilia. It involves mixing plasma from the test person and a typical control sample and assessing the results.
- Tests to measure coagulation factors and inhibitor levels: Low activity levels of clotting factor VIII and signs of clotting factor VIII inhibitor may suggest a person has acquired hemophilia.
People who are most susceptible to acquired hemophilia include the elderly and those who are about to or have recently given birth.
People with rheumatoid arthritis have swollen and tender small joints.
- pain, aching, stiffness, or tenderness in more than one joint
- the same symptoms on both sides of the body
- weight loss
Diagnosing this condition involves reviewing the above symptoms, carrying out a physical examination, taking X-rays, and doing lab tests.
The main goals of managing acquired hemophilia disease are:
- getting the bleeding under control
- eradicating the inhibitor
- resolving the underlying cause of disease, where possible
As acquired hemophilia is rare, developing most therapies involves using anecdotal or small case reports. Treatments are highly specialized and depend on many factors, including age, overall health, tolerance for certain medications, and the underlying cause.
Some medications include:
- desmopressin to help maintain healthy blood, kidneys, and blood pressure
- blood clotting medications, such as
- factor VIII concentrate
- activated prothrombin complex concentrate
- recombinant activated human factor VII
- corticosteroids, an anti-inflammatory medication
- cyclophosphamide, which doctors use in chemotherapy and to suppress the immune system
- rituximab, a monoclonal antibody medication
When a person’s acquired hemophilia has links to another autoimmune condition, such as rheumatoid arthritis, doctors most commonly write a combined prescription for methylprednisolone and cyclophosphamide. Both medications help prevent the overactive immune system from attacking healthy cells.
Research from 2018 shows that acquired hemophilia is a life threatening condition, and
When doctors diagnose a person with acquired hemophilia early and take action to treat the condition sooner, a person’s outlook can be more positive. However, some healthcare professionals can miss or misdiagnose the condition.
Additionally, treatment options come with risks. Therefore, people with acquired hemophilia need regular monitoring from their doctors.
Hemophilia may be congenital or acquired. People with acquired hemophilia can have other autoimmune conditions, such as rheumatoid arthritis, at the same time.
Many laboratory tests can help diagnose and treat this condition. Doctors prescribe medications to people with the following goals: controlling bleeding, eradicating the inhibitor, and treating the underlying disease where possible.
The sooner doctors diagnose and treat a person with acquired hemophilia secondary to rheumatoid arthritis, the better their chances of survival.