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Genetic and Rare Diseases Information Center (GARD)

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Evans syndrome


Other Names for this Disease

  • Autoimmune hemolytic anemia and autoimmune thrombocytopenia
  • Evan syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have been recently diagnosed with Evans syndrome. Can you provide me with information about this condition, including treatment options and mortality rates?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might Evans syndrome be treated?

The best treatment options for Evans syndrome depend on many factors, including the severity of the condition; the signs and symptoms present; and each person's response to certain therapies. For example, people who need to be hospitalized due to severe anemia or thrombocytopenia are often treated with blood transfusions followed by therapy with corticosteroids or intravenous (IV) immune globulin. Other treatment options include immunosuppressive drugs.[1] Most affected individuals respond to these treatments; however, relapse is frequent.[2]

In people who do not respond to standard treatments, therapy with rituximab or splenectomy may be considered. Some people with Evans syndrome respond well to rituximab treatment and experience an extended period of remission, while others have little to no response.[2] People undergoing splenectomy may show immediate improvement; however, relapses are common and, in most cases, occur within 1-2 months after the procedure.[1]

For cases that are very severe and difficult to treat, stem cell transplantation may be used to provide a long-term cure.[2] Autologous and allogeneic stem cell transplantation have been used in a small number of patients (14 patients aged 5-52 years), with mixed results.[1]
Last updated: 11/17/2014

Are statistics available regarding morbidity and mortality for Evans syndrome? 

Evans syndrome has a chronic, relapsing, and sometimes fatal course. In a 3-year follow-up study of 42 people with Evans syndrome (ages 4 months to 19 years), 3 people (7%) died; 20 people (48%) had active disease and remained on some treatment; and 5 people (12%) had persistent disease but were not receiving any treatment. Fourteen people (33%) had no evidence of disease for 1.5 months to 5 years (median 1 year).[3]
Last updated: 11/16/2014

What is the long-term outlook for people with Evans syndrome?

The long-term outlook for people with Evans syndrome can vary. Some affected people may experience periods of long remission in which the signs and symptoms of the condition disappear or become less severe. Others have chronic problems with no remissions.[4]

Those with Evans syndrome rarely do well without treatment. Even with treatment, response to therapy can be variable and often disappointing. Recurrences of thrombocytopenia and anemia are common, as are episodes of hemorrhage (bleeding) and serious infections.[1] People with Evans syndrome have a greater tendency to develop other autoimmune disorders such as systemic lupus erythematosus (SLE), lymphoproliferative disorders, or primary immunodeficiencies.[4][5] Evans syndrome is sometimes fatal so careful monitoring by a physician who is familiar with this condition is important.[1]
Last updated: 11/16/2014

References
Other Names for this Disease
  • Autoimmune hemolytic anemia and autoimmune thrombocytopenia
  • Evan syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.