Evans Syndrome

What is Evans syndrome?

Evans syndrome is an autoimmune disease that destroys certain types of blood cells in your body, causing you not to have enough. Having abnormally low levels of certain blood cells is called cytopenia.

Most people with Evans syndrome develop a low red blood cell count (anemia) and low platelet count (thrombocytopenia). Some are also low in a type of white blood cell called neutrophils (neutropenia).

These conditions affect the amount of oxygen in your blood, how well your blood clots and how well your body fights infections. They can make you weak and tired, prone to bleeding and prone to illness.

Evans syndrome can be mild to severe and affect you a little or a lot. How much it affects you can vary over the course of your life. There’s no cure, but treatment can help manage the symptoms and effects.

What are the symptoms of Evans syndrome?

Evans syndrome symptoms can vary widely, depending on which types of cytopenia you have and how advanced they are. Most people develop anemia or thrombocytopenia first, followed by others later.

Early symptoms of anemia (low red blood cells) may include:

• Unusual tiredness (fatigue).
• Pale complexion (pallor).
• Lightheadedness or dizziness.
• Shortness of breath (dyspnea).
• Rapid heartbeat (tachycardia).
• Unusual heartbeat or heart palpitations.

Early symptoms of thrombocytopenia (low platelets) may include:

• Tiny red spots of bleeding under the skin (petechiae).
• A rash of purple patches on your skin (purpura).
• Unexplained bruises (ecchymosis).
• Heavily bleeding gums or nosebleeds.
• Heavy menstrual bleeding.
• Blood in your poop or in your pee.

Symptoms of neutropenia (low neutrophils) may or may not occur later. These may include:

• Fever.
• Mouth sores (ulcers).
• Frequent colds, flus and stomach bugs.
• Recurring ear infections.
• Persistent fungal infections.
• Urinary tract infections (UTIs).

Other signs and symptoms of Evans syndrome, which may occur off and on, include:

• Swollen lymph nodes in your neck.
• Enlarged spleen.
• Enlarged liver.
• Jaundice.

What causes Evans syndrome?

Evans syndrome is an autoimmune disease, which means that your immune system malfunctions. It mistakes part of your body (in this case, certain blood cells) for a foreign invader and launches an attack.

Special immune cells called B cells create antibodies that recognize, seek and destroy certain types of blood cells. They usually start by targeting one type, and then begin targeting other types, as well.

This process causes you to eventually develop at least two and sometimes three cytopenias — deficits of certain types of blood cells. Having all three is called pancytopenia. The three types include:

• Autoimmune hemolytic anemia (AIHA). Anemia is a deficit of red blood cells. Hemolytic anemia happens when your body destroys red blood cells faster than your body can reproduce them.
• Immune thrombocytopenia (ITP). Thrombocytopenia is a deficit of platelets. Immune thrombocytopenia happens when your immune system clears platelets from your circulation.
• Autoimmune neutropenia (AIN). Neutropenia is a deficit of neutrophils, which are a type of white blood cell. AIN is when your immune system attacks and removes your neutrophils.

Cytopenia can happen for different reasons. Sometimes, your bone marrow just doesn’t produce enough blood cells. But autoimmune cytopenia happens because your immune system destroys your blood cells.

What triggers Evans syndrome?

Scientists don’t know why autoimmune diseases occur. But they’ve noticed that they often seem to develop after a serious illness or event that stressed your immune system. These are called triggers.

Having one type of autoimmune disease also makes you statistically more likely to get another one. It’s as if the dysfunction in your immune system spreads from one part of your body to another.

Evans syndrome can either occur on its own (primary Evans syndrome) or as a secondary condition in combination with another condition (secondary Evans syndrome). Some of these conditions include:

• Systemic lupus erythematosus (lupus).
• Common variable immunodeficiency.
• Selective IgA deficiency.
• Sjögren’s syndrome.
• Non-Hodgkin lymphoma.
• Chronic lymphocytic leukemia.
• Chronic hepatitis C.
• Chronic HIV infection.

If you have primary Evans syndrome, it may have developed after a temporary illness, or there may be no obvious trigger. Scientists classify Evans syndrome as idiopathic, meaning there’s no direct cause.

What are the complications of Evans syndrome?

Not everyone has complications with Evans syndrome. Some people only have mild symptoms. But in severe cases, cytopenias (blood cell deficits) can have life-threatening effects. For example:

• Not having enough red blood cells to carry oxygen leaves you with a lack of oxygen in your blood (hypoxemia). This is hard on your heart and can lead to cardiovascular disease and heart failure.
• A lack of platelets in your blood prevents it from clotting effectively, making you prone to bleeding too easily and too fast. This could quickly lead to severe blood loss.
• A lack of neutrophils in your blood to help fight infections means you’re more likely to get more infections, and they’re more likely to become severe and spread. This could lead to sepsis.

How is Evans syndrome diagnosed?

Blood tests diagnose Evans syndrome. You might have a blood test if you develop symptoms of cytopenia, or you might have one as part of a routine health checkup, including a blood cell count.

If your complete blood count (CBC) reveals low levels of red blood cells, platelets or neutrophils, your healthcare team will investigate further by conducting additional tests on your blood sample.

For example, they might look for high levels of antibodies associated with Evans syndrome in your blood. They’ll also test for other possible causes of cytopenia, including certain infectious diseases and cancer.

Healthcare providers diagnose Evans syndrome only by ruling out other causes. In some cases, they might want to conduct other tests to help rule out conditions, like a CT scan (computed tomography scan) or a bone marrow biopsy.

Is there a cure for Evans syndrome?

There’s no cure for Evans syndrome or any autoimmune disease. Healthcare providers treat autoimmune diseases with medications that turn the volume down on your immune response.

They can also treat the symptoms or effects of your condition separately. For example, if your blood cell counts get too low, your healthcare provider can boost them periodically with blood transfusions.

Like many chronic (long-term) autoimmune conditions, Evans syndrome may get better or worse at different times. Sometimes, symptoms seem to go away, but then they return or worsen.

You might need to try different treatments throughout your life because their effectiveness can change. Your provider will work to prevent the most serious complications of Evans syndrome.

What is the treatment for Evans syndrome?

Standard treatment for Evans syndrome includes immunosuppressant medications and blood transfusions when necessary. Immunosuppressants act to suppress your immune system’s attack.

Medications include:

• Corticosteroids, like prednisone. This is the most typical first-line treatment.
• Intravenous immunoglobulin therapy (IVIg). This is an infusion of human antibodies delivered into your bloodstream. The new antibodies can overwhelm and confuse the autoimmune ones.

Your provider might try other treatments if you don’t respond well to the standard ones, including:

• Monoclonal antibodies like rituximab. These are lab-made antibodies that can sometimes inhibit an autoimmune response. Your provider might suggest these if other medications don’t work.
• Splenectomy. In severe cases, your provider might suggest a procedure to remove your spleen. Your spleen removes retired blood cells from circulation, so getting rid of it can slow that process.

Complications or side effects of treatment

Taking immunosuppressants can reduce your immune response to your autoimmune disease, but it also reduces your immune response to infections. You might need to take special precautions to avoid getting sick.

Your provider might also recommend certain vaccines beyond the ones you may have already received. When you’re immunocompromised, you become vulnerable to diseases that you normally wouldn’t be.

What is life expectancy with Evans syndrome?

Evans syndrome won’t necessarily affect your life expectancy. Some people have only mild or occasional symptoms. But certain complications, like bleeding, heart disease and infections, can be life-threatening.

If you have secondary Evans syndrome with another chronic condition, this also affects your life expectancy. Your other condition may come with its own set of life-threatening complications.

What is the outlook for Evans syndrome?

People with Evans syndrome can have a wide range of experiences. Symptoms can be mild to severe. For some, they seem to disappear (go into remission). And for others, they reappear later (relapse).

Most people will need some treatment and regular checkups afterward. Treatment often works well, but not always. Sometimes, it doesn’t seem to work as well over time. You might need to try other treatments.

How do I take care of myself while living with Evans syndrome?

If you have Evans syndrome, it’s important to keep up with your regular healthcare check-ins. Even if you feel well, your provider will want to keep an eye on your condition and catch it if it starts to decline.

You should also check in with yourself regularly to notice if you have any new or emerging symptoms. If you catch a cold that doesn’t seem to get better, or develop uncontrollable bleeding, call your provider.

What questions should I ask my healthcare provider?

You might want to ask your provider:

• Which blood cells am I low in?
• How low are they?
• What symptoms or warning signs should I watch out for?
• How will I know if the treatment is working or not?
• What happens if the treatment doesn’t work?
• When should I schedule a follow-up appointment?

A note from Cleveland Clinic

Evans syndrome is a rare condition that you might not have heard of before. The symptoms can be strange and worrying. Piecing together what’s going on and what it means can be an unsettling process.

Evans syndrome is lifelong, but it doesn’t always stay the same. You should expect to feel better, at least sometimes. However you’re feeling, your healthcare team will be there to provide the care you need.