Evans syndrome (2137)

What is Evan’s syndrome? 

It is a type of autoimmune disease. It is when your immune system attacks two or more different types of your blood cells. The blood cells can be attacked at the same time. Or one type of cell may be attacked after another. You will be seen by a hematologist. They are providers that work with blood disorders.

The blood cells that are usually affected are:

• the red blood cells (the cells that carry oxygen).
• the platelets (the cells that help you stop bleeding).
• the white blood cells (the cells that fight infection).

What causes it?  

The cause of Evan’s syndrome is not known.  Sometimes it can be from a change in the genes. This change makes the person more likely to have a problem with their immune system.  Treatment may depend on if the type of genetic change can be found.

What are the symptoms? 

Symptoms are different for each person.  It depends on which type of blood cell is affected.

If your white blood cells are being attacked, you might have:

• mouth sores
• fevers
• more infections 

If your red blood cells are being attacked, you might have:

• pale skin
• jaundice (yellow skin and eyes)
• fatigue (extremely tired)
• weaknessdark urine (pee)
• trouble breathing

If your platelets are being attacked, you might have:

• bruising
• bleeding, like bloody noses or heavy periods
• petechiae (tiny red dots on the skin)

How is it diagnosed?

Evan’s syndrome is diagnosed when a child has two or more low blood cell numbers caused by the immune system. The hematology team will do blood tests to help diagnose it. The blood tests will also help understand how the immune system is working. Your provider will talk with you about which tests are needed. 
The team may also need to do these tests:

• Bone marrow test
• Chest X-ray
• Ultrasound of the stomach
• CT scan
• Genetic tests

What is the treatment?

The treatment for Evan’s syndrome will depend on the symptoms.  Many children are given a steroid medicine, like prednisone.  This helps slow down the attack from the immune system. Other medicines can be given depending on the type of cell being attacked. Your child may need to take more than 1 type of medicine. Some medicines used include:

• intravenous immunoglobulin (IVIG)
• medicine to increase platelet count
• monoclonal antibodies
• medicine to slow down the immune system
• medicine to increase red blood cells
• medicine to increase white blood cells

Will my child need treatment for their whole life?

The treatment for Evan’s syndrome is different for each child. There are usually times that your child will feel well. This is called remission. There may be times when they feel sick. This is called recurrence.  The recurrence happens when the immune system starts destroying the cells again.

Your child will need to see their provider about this for their whole life. If they have a genetic problem, there may be certain treatment or monitoring that your child needs.  This can be hard. Know that your team is here to support you.  

Children’s Wisconsin KIDDs (Kids with Immune Dysregulation Disorders) Program

Children with Evan’s syndrome are managed by the Children’s Wisconsin KIDDs Program. This team includes many different specialists that work together. The team works with each child to decide the best diagnosis and treatment.