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Evans' syndrome

Also known as: Evans syndrome

Background

Evans' syndrome is a rare autoimmune condition (where the immune system attacks the body's own cells). In this condition, the body makes antibodies that destroy the red blood cells, platelets (small bodies within the blood that help it clot) and white blood cells (which help fight off infection). Destruction of the platelets causes immune thrombocytopenia (low levels of platelets) and autoimmune haemolytic anaemia (low levels of red blood cells). Generally the red cells and the platelets are the cells most affected in Evans' syndrome.

Evans' syndrome affects people of all ages, but is usually first diagnosed in young children.

Credits

Last updated September 2014 by Dr AB Provan, Consultant Haematologist and Senior Lecturer in Haematology, Barts & The London School of Medicine and Dentistry, London, UK.

What are the symptoms?

There are a number of features found in people with Evans' syndrome, which are caused by the destruction of red blood cells, white blood cells and platelets. As red blood cells carry oxygen in the blood, low levels may cause:

  • pallor (paleness), fatigue (tiredness) and light-headedness.

With low platelets, those with Evans' syndrome may have:

  • purpura (patches of purplish discoloration resulting from leaking of blood into the skin)
  • petechiae (tiny localised haemorrhages from the small blood vessels just beneath the surface of the skin)
  • ecchymoses (bruises)

A reduction in white blood cells may cause an increased likelihood of infections.

 

What are the causes?

The cause of Evans' syndrome has not been identified but it is known that some people diagnosed with the syndrome have associated disorders such as systemic lupus erythematosus (see entry Lupus), and other autoimmune disorders.

 

How is it diagnosed?

Diagnosis of the syndrome is made after other possible conditions are ruled out. These include rheumatological conditions (where the immune system is overactive and attacks the joints), malignancies (a tumour that spreads) that first present with autoimmune cytopenias (deficiency of some specific cellular elements of the blood) and infections. Blood samples will be taken to measure the levels of white blood cells, red blood cells and platelets.

How is it treated?

Closely monitoring the patient's full blood count (FBC; in the USA it is called the complete blood count) is crucial to the management of the condition. Treatment involves medication such as steroids or other immunosuppressives so that antibodies to red blood cells and platelets are no longer produced. Splenectomy, removal of the spleen by surgery, can have short-lasting positive effects. The spleen is the place in the body where red blood cells and platelets are broken down, so removing it helps prevent this. Intravenous immune globulin or IVIG may be used. This works by confusing the immune system which reduces the rate of red cell and platelet breakdown.

Transfusion of blood products is carried out in crisis situations to help stabilise the affected individual, but is not a long-lasting solution as these cells are usually destroyed very quickly by the antibodies the person produces.

Inheritance patterns and prenatal diagnosis

Is there support?

There is no support group for Evan's syndrome in the UK. Cross referrals to other medical information entries on our website are intended to provide relevant support for those particular features of the disorder. Organisations identified in those entries do not provide support specifically for Evan's syndrome.

Families can use Contact a Family's freephone helpline for advice, information and, where possible, links to other families. Contact a Family's web-based linking service can be accessed at community.cafamily.org.uk

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