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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
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How might Evans syndrome be treated?

Evans syndrome is generally considered to be a chronic condition because it is characterized by periods of exacerbation and remission. Initial attempts to treat this condition usually involve corticosteroids and/or intravenous immunoglobulin. Most affected individuals respond to these treatments; however, relapse is frequent.[1]

Other options for treatment include immunosuppressive drugs, especially cyclosporine or mycophenolate mofetil; vincristine; danazol or a combination of these medications. Treatment with danazol induces remission in the majority of cases, although remission often lasts less than 12 months and the long-term effects are unclear. Splenectomy may also be considered, although benefits are often short-lived.[1]

Rituximab is another medication used when affected individuals have not responded to other forms of therapy. Some individuals with Evans syndrome have responded well to rituximab treatment and experienced an extended period of remission; however others have had little to no response. The complications associated with the use of rituxumab have been limited in studies performed to date.[2]

For cases that are very severe and difficult to treat, stem cell transplantation (SCT) may be used to provide a long-term cure. There is some data that suggests allogeneic SCT may be superior to autologous SCT, but both carry serious risks. Reports have also indicated that using a regimen called "reduced-intensity conditioning" may be beneficial in individuals who receive bone marrow transplantation.[1]

Last updated: 9/22/2011

  1. Norton A, Roberts I. Management of Evans syndrome. Br J Hematol. January 2006; Accessed 2/6/2009.
  2. Mathew P. Evans Syndrome. eMedicine. November 19, 2009; Accessed 9/22/2011.

Clinical Trials & Research for this Disease

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