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Diseases

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
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Treatment

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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

References
  1. Prasad Mathew, MBBS, DCH. Evans Syndrome. Medscape. January 8, 2014; http://emedicine.medscape.com/article/955266-overview#a0101. Accessed 11/16/2014.
  2. Norton A, Roberts I. Management of Evans syndrome. Br J Hematol. January 2006; 132(2):125-137. http://www.ncbi.nlm.nih.gov/pubmed/16398647?dopt=AbstractPlus. Accessed 2/6/2009.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Evans syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
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.