Definition

What is Evans Syndrome?

Evans syndrome is a disorder in which the body’s immune system produces antibodies that mistakenly destroy red blood cells, platelets and sometimes certain white blood cell known as neutrophils. This leads to abnormally low levels of these blood cells in the body (cytopenia). The premature destruction of red blood cells (hemolysis) is known as autoimmune hemolytic anemia or AIHA.

How common is Evans Syndrome?

Evans syndrome is rare. The disorder can affect children or adults. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Evans Syndrome?

The common symptoms of Evans Syndrome are:

  • Low platelet counts (thrombocytopenia):
    • Increased bruising
    • Increased bleeding: blood blisters in the mouth, nosebleeds, gum bleeding, blood in urine or stool
    • Tiny red dots on the skin called petechiae
  • Anemia:
    • Fatigue or tiredness
    • Shortness of breath
    • Elevated heart rate (tachycardia)
    • Yellowing of the skin (jaundice) or eyes (scleral icterus)
    • Dark urine (tea or coca-cola colored)
  • Low neutrophil count (neutropenia):
    • Fever
    • Infections of skin or mouth

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Evans Syndrome?

The exact cause of Evans syndrome is not known; however, it is known that Evans syndrome is a disorder of the immune system. The immune system is a network of cells, tissues, and organs that work together to defend the body against germs (foreign substances). The immune system normally responds to foreign substances by producing specialized proteins, called antibodies, that target foreign invaders for eventual destruction by white blood cells. Disorders of the immune system like Evans syndrome occur when the immune system produces antibodies that mistakenly attack healthy tissue, specifically red blood cells, platelets, and white blood cells.

Evans syndrome may occur in combination with another disorder as a secondary condition. Disorders that can be associated with Evans syndrome include but are not limited to: autoimmune lymphoproliferative syndrome (ALPS), lupus, antiphospholipid syndrome, Sjogren syndrome, common variable immunodeficiency, IgA deficiency, certain lymphomas, and chronic lymphocytic leukemia.

Risk factors

What increases my risk for Evans Syndrome?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Evans Syndrome diagnosed?

As most people with Evans Syndrome already carry one of the diagnoses, the presentation of another one equals Evans Syndrome.  For example, if you have been diagnosed with ITP and develop anemia, your physician will need to determine the cause of your anemia.  If your anemia is found to be due to AIHA, you will be diagnosed with Evans Syndrome.

Because these disorders affect your blood counts, a complete blood count (CBC), is the first step in the work up.  Your physician is looking evidence of anemia (low hemoglobin), thrombocytopenia (low platelet count) or neutropenia (low neutrophil count, a type of white blood cell).  Your blood will be examined under a microscope to try to identify the cause.  ITP and AIN are diagnoses of exclusion meaning there is no one specific diagnostic test.  Your physician must rule out other causes first.  AIHA is confirmed by multiple tests, most specifically a test called DAT (direct antiglobulin test).  The DAT looks for evidence that the immune system is attacking the red blood cells.

How is Evans Syndrome treated?

There is a long list of possible treatments.  Treatments are directed at the specific blood cell affected and whether you have any symptoms (active bleeding, shortness of breath, elevated heart rate, infection):

  • Steroids: Medications like prednisone have been used for years in various autoimmune disorders. They are the first line treatment for AIHA and are also used in ITP.  Unfortunately, if you have Evans Syndrome, you may require steroids for long periods of time which may result in other issues like high blood pressure (hypertension) and elevated blood sugars (diabetes).  For this reason, you physician may look for alternative therapies.
  • Intravenous immunoglobulin (IVIG): IVIG is the first-line treatment for ITP. Essentially, the IVIG distracts your immune system temporarily so that the platelets are not destroyed as rapidly.  IVIG is not as effective in AIHA or AIN.
  • Splenectomy: The spleen is the main location of the destruction of the red blood cells, platelets, and neutrophils in Evans Syndrome. In some patients, surgical removal of the spleen might improve blood counts, but this may be only temporary.
  • Rituximab: Rituximab in a medication called a monoclonal antibody. It reduces your B-lymphocytes (a white blood cell that makes antibodies), which can improve your blood counts.
  • G-CSF (filgrastim): G-CSF is s a medication used to stimulate the bone marrow to make more neutrophils.  It is sometimes used to increase the neutrophil count in AIN, particularly if you have an infection.
  • Immunosuppressive medications: These medications inhibit the immune system. These would include medications like mycophenolate motel (MMF), azathioprine (Immuran), tacrolimus (Prograf).  ​

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Evans Syndrome?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: May 22, 2018 | Last Modified: May 22, 2018

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