Other Names for this Disease
- Autoimmune hemolytic anemia and autoimmune thrombocytopenia
- Evan syndrome
Your QuestionI have been recently diagnosed with Evans syndrome. Can you provide me with information about this condition, including treatment options and mortality rates?
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Questions on this page
- What is Evans syndrome?
- What causes Evans syndrome and can it be inherited?
- What are the signs and symptoms of Evans syndrome?
- How might Evans syndrome be treated?
- Are statistics available regarding morbidity and mortality for Evans syndrome?
- What is the prognosis for individuals with Evans syndrome?
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.
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.
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.
In a 3-year follow-up study of 42 individuals with Evans syndrome (ages 4 months to 19 years), 3 individuals (7%) had died, 20 (48%) had active disease and remained on some treatment, and 5 (12%) had persistent disease but were not receiving any treatment.
- What is Evans Syndrome?. Evans Syndrome Research and Support . http://www.evanssyndrome.org/#evans. Accessed 9/21/2011.
- Mathew P. Evans Syndrome. eMedicine. November 19, 2009; http://emedicine.medscape.com/article/955266-overview. Accessed 9/22/2011.
- Norton A, Roberts I. Management of Evans syndrome. Br J Hematol. January 2006; http://www.ncbi.nlm.nih.gov/pubmed/16398647?dopt=AbstractPlus. Accessed 2/6/2009.
- Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009; http://bloodjournal.hematologylibrary.org/content/114/15/3167.full. Accessed 9/22/2011.