Estimates are that fewer than 5,000 people in the United States have Evans syndrome, a rare but serious condition that impacts blood cells and can lead to fatigue, dizziness, pale or bruised skin, and purple or red spots on the skin.

The condition is named after Robert Evans, who first described it in medical literature in 1951. Because the condition is so rare, you might not have heard of it.

This article explains Evans syndrome, its symptoms, and treatment.

Evans syndrome is the combination of autoimmune hemolytic anemia (AIHA) with immune thrombocytopenia (ITP) or autoimmune neutropenia (AIN).

Each condition impacts the blood differently:

  • The immune system destroys red blood cells in AIHA.
  • In ITP, the immune system attacks platelets.
  • In individuals with AIN, the immune system attacks neutrophils.

A person usually develops one condition at a time, but occasionally they can occur at the same time. Experts are unsure of the exact cause of Evans syndrome. It does not appear to run in families.

Still, illness, infection, and other health conditions may lead to Evans syndrome.

Evans syndrome may appear by itself as a primary disorder or with other disorders as a secondary disorder. Determining this is important because it can affect treatment and prognosis.

No one specific test is used to diagnose Evans syndrome. Instead, a doctor may conduct various tests to rule out other conditions. This can include:

The exact symptoms that indicate Evans syndrome vary from person to person, along with the combination of conditions involved.

When red blood cells are low, a person may show signs of anemia, such as:

Low platelet levels mean blood may not clot properly, and bleeding under the skin may occur. This can result in:

  • petechiae (tiny purple or red spots on the skin)
  • purpura (raised purple spots on the skin)
  • ecchymosis (larger purple blotches on the skin)
  • easily bruised skin

Low neutrophil levels can result in a person getting sick more easily. Frequent fevers, illnesses, and sores or ulcers may all result.

People with Evans syndrome may also have an enlarged spleen, liver, or lymph nodes that show in scans.

Evans syndrome is a very serious illness. One study reported a median survival of 7.2 years with Evans syndrome. Those with secondary Evans syndrome had a 5-year survival rate of 38%.

The leading causes of death for those with Evans syndrome are bleeding, infections, and hematological cancer.

The precise ratio of childhood AIHA and Evans syndrome has not yet been determined. However, it’s estimated that 2 to 5 cases per 100,000 children younger than 18 have ITP.

Individuals with Evans syndrome can go through periods of remission; however, there is no known cure for this disease.

One study looking at the effects of Evans syndrome in children found that 69% of children required one or more second-line immune treatments, and 10% died at the age of 14.3 years.

There is no cure for Evans syndrome. As a result, doctors focus on relieving symptoms and increasing blood cell counts. Treatment will typically be individualized based on:

  • age
  • symptoms
  • blood count
  • overall health

What are the treatment options for Evans syndrome?

  • corticosteroids to help lower immune system activity
  • with antibodies from healthy individuals to help prevent the immune system from attacking the body
  • immunosuppressive drugs to lower the activity level of the immune system
  • Rituximab, a newer medication designed to avoid some of the side effects found with immunosuppressive drugs)
  • hematopoietic stem cell transplantation to help reset the immune system

Spleen removal or splenectomy may be recommended in some cases. However, the effectiveness varies, and symptoms usually return after the procedure, so this treatment option is usually delayed as long as possible.

How long can a dog live with Evans syndrome?

Life expectancies for dogs with Evans syndrome range greatly. The prognosis frequently depends on how well they respond to treatments. Dogs with secondary Evans syndrome associated with infections or cancer may also have a shorter life span depending on the primary cause.

What are the symptoms you might see with Evans Syndrome?

Exact symptoms vary based on the underlying conditions that combine to form an individual’s Evans syndrome. Individuals may experience signs of anemia from low red blood cell counts. Also, those with impaired platelets may show evidence of increased bruising. Those with low neutrophil levels may have frequent fevers and illnesses.

Can radiology treat Evans syndrome?

As a rare disorder, Evans syndrome can be difficult to diagnose properly. X-rays and ultrasounds can be combined with blood tests to rule out other conditions.

Evans syndrome is a rare autoimmune disorder that involves multiple underlying blood conditions. Because there is no known cure, treatment focuses on addressing an individual’s symptoms and blood count.

If you believe you are showing signs of Evans syndrome (or any other disorder), it’s important to talk with your doctor. They can determine whether any diagnostic tests or procedures are needed. They can also provide referrals and treatment plan recommendations.