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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.

Overview

What is Evans syndrome?

What are the signs and symptoms of Evans syndrome?

What causes Evans syndrome?

Is Evans syndrome inherited?

How might Evans syndrome be treated?


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

What is Evans syndrome?

Evans syndrome is a very rare autoimmune disorder in which the immune system destroys the body's red blood cells, white blood cells and/or platelets.[1] Affected people often experience thrombocytopenia (too few platelets) and Coombs' positive hemolytic anemia (premature destruction of red blood cells). Signs and symptoms may include purpura, paleness, fatigue, and light-headedness.The exact cause of this condition is unknown. 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.[2]
Last updated: 11/17/2014

What are the signs and symptoms of Evans syndrome?

The signs and symptoms of Evans syndrome vary from person to person and largely depend on which type(s) of blood cells are affected (i.e. platelets, white blood cells, or red blood cells). If a person does not have enough healthy red blood cells (anemia), they may experience weakness, fatigue, paleness, light-headedness, and shortness of breath. Low platelets can cause easy or unexplained bruising; prolonged bleeding from small cuts; and purpura. People with low white blood cells may be more susceptible to infections.[2]

Many people with Evans syndrome go through periods of remission in which the signs and symptoms of the condition temporarily disappear or become less severe.[2]
Last updated: 11/16/2014

What causes Evans syndrome?

Evans syndrome is believed to be a condition of immune system dysregulation. However, the exact cause of the condition is unknown.[2]
Last updated: 11/16/2014

Is Evans syndrome inherited?

Evans syndrome is not thought to be inherited in most cases and rarely occurs in more than one person in a family.[3][4][5]
Last updated: 11/16/2014

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.[2] Most affected individuals respond to these treatments; however, relapse is frequent.[6]

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.[6] People undergoing splenectomy may show immediate improvement; however, relapses are common and, in most cases, occur within 1-2 months after the procedure.[2]

For cases that are very severe and difficult to treat, stem cell transplantation may be used to provide a long-term cure.[6] Autologous and allogeneic stem cell transplantation have been used in a small number of patients (14 patients aged 5-52 years), with mixed results.[2]
Last updated: 11/17/2014

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.[1]

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.[2] People with Evans syndrome have a greater tendency to develop other autoimmune disorders such as systemic lupus erythematosus (SLE), lymphoproliferative disorders, or primary immunodeficiencies.[1][7] Evans syndrome is sometimes fatal so careful monitoring by a physician who is familiar with this condition is important.[2]
Last updated: 11/16/2014

References
  1. What is Evans Syndrome?. Evans Syndrome Research and Support . http://www.evanssyndrome.org/#evans. Accessed 9/21/2011.
  2. Prasad Mathew, MBBS, DCH. Evans Syndrome. Medscape. January 8, 2014; http://emedicine.medscape.com/article/955266-overview#a0101. Accessed 11/16/2014.
  3. Ahmed FE, Albakrah MS. Neonatal familial Evans syndrome associated with joint hypermobility and mitral valve regurgitation in three siblings in a Saudi Arab family.. Ann Saudi Med. May 2009; 29(3):227-230. Accessed 11/16/2014.
  4. Ahmed FE, Qureshi IM, Wooldridge MA, Pejaver RK.. Hereditary spastic paraplegia and Evans's syndrome. Acta Paediatr. July 1996; 85(7):879-881. Accessed 11/16/2014.
  5. McLeod AG, Pai M, Carter RF, Squire J, Barr RD.. Familial Evans syndrome: a report of an affected sibship.. J Pediatr Hematol Oncol.. May-June 1999; 21(3):244-247. Accessed 11/16/2014.
  6. 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.
  7. 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. October 2009; 114(15):3167-3172. http://bloodjournal.hematologylibrary.org/content/114/15/3167.full. Accessed 9/22/2011.


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.