Cookies help us deliver our services. By using our services, you agree to our use of cookies. Learn more

Evans Syndrome

What is Evans Syndrome?

Evans syndrome is a rare autoimmune disorder, meaning that the body’s immune system attacks and damages healthy tissue. In the case of Evans syndrome, blood cells are targeted by the immune system. Blood cells include red blood cells, responsible for the transport of oxygen, white blood cells with an immune function, and platelets which are mostly involved in blood clotting to stop bleeding. As a result of Evans syndrome, affected individuals may have blood cells that are inadequate in number or in function. The exact cause of Evans syndrome is unknown. No genes have been identified and in most cases, it does not appear to be inherited.

 

Synonyms

  • Evans Syndrome

Evans syndrome is a rare autoimmune disorder, meaning that the body’s immune system attacks and damages healthy tissue. In the case of Evans syndrome, blood cells are targeted by the immune system. Blood cells include red blood cells, responsible for the transport of oxygen, white blood cells with an immune function, and platelets which are mostly involved in blood clotting to stop bleeding. As a result of Evans syndrome, affected individuals may have blood cells that are inadequate in number or in function. The exact cause of Evans syndrome is unknown. No genes have been identified and in most cases, it does not appear to be inherited.

Acknowledgement of Evans Syndrome has not been added yet.

The exact prevalence of Evans syndrome is unknown. As it is the case with many autoimmune conditions, females appear to be affected more commonly than men, with a female to male ratio of 3:2. Evans syndrome is also more common during childhood but it can occur in adults as well.

 

Name Abbreviation
Evans Syndrome ES

Evans syndrome can be classified as idiopathic (unknown cause) or secondary to another condition. The exact cause of idiopathic Evans syndrome is unknown, although it is believed to be a result of the dysregulation of the immune system. The deficiency of certain molecules involved in the regulation of the immune system is among the possible explanations. Secondary Evans syndrome has been associated with other conditions including autoimmune lymphoproliferative syndrome, systemic lupus erythematosus, and autoimmune hepatitis, all of which are autoimmune conditions targeting different organs, as well as chronic lymphocytic leukemia which is a type of cancer affecting the blood and the bone marrow.

Symptoms of Evans syndrome can vary among individuals and depend on the specific type of blood cell that is affected. If red blood cells are damaged, hemolytic anemia can occur which is a condition in which red blood cells are produced but are destroyed faster than in healthy individuals. Symptoms of hemolytic anemia include an abnormally pale-looking skin, fatigue, weakness, dizziness, shortness of breath, and jaundice. Jaundice is a condition caused by high levels of a substance called bilirubin which causes the white of the eye and the skin to appear yellow. 

 

When platelets are affected, thrombocytopenia or abnormally low levels of platelets result. Thrombocytopenia leads to easy bruising and bleeding as blood does not effectively clot. In addition, red, brown, or purple dots or larger spots (petechiae and purpura) that are often clustered together may appear on the skin as a result of bleeding. 

 

If white blood cells are affected, the individual becomes prone to recurrent infections. They may also notice sores inside their mouth or experience fever. 

 

In each individual, only one type of blood cell might be affected, or multiple blood cell types could be damaged concurrently. Furthermore, affected individuals may experience the symptoms over a long period of time or as intermittent episodes of relapse and remission. 

 

To diagnose Evans syndrome, a number of blood tests are performed to look for low red blood cell count, thrombocytopenia, or an increase in certain other markers such as bilirubin. If test results indicate Evans syndrome, other tests are performed to determine whether it is idiopathic or secondary Evans syndrome. If all possible underlying conditions have been ruled out, idiopathic Evans syndrome is diagnosed. If additional testing indicates an underlying condition, secondary Evans syndrome is diagnosed.

The diagnosis of Evans syndrome involves taking a blood sample to check for features of autoimmune hemolytic anemia, a condition in which red blood cells are attacked and destroyed by the immune system. A direct antiglobulin test (DAT) is among such tests. DAT is a test that can determine whether hemolytic anemia is caused by the attack of the immune system. This is achieved by looking for defensive proteins (antibodies) that are attached to red blood cells, indicating that the immune system is targeting these cells. Other tests may be done to determine whether there is an underlying cause. For example, a blood sample may be tested for the presence of certain markers that indicate other autoimmune conditions such as autoimmune hepatitis, Graves disease, or rheumatoid arthritis. 

 

Treatment of Evans syndrome usually starts with corticosteroids which are a group of anti-inflammatory drugs that can also lower the activity of the immune system. Although inflammation is a natural immune process, excessive inflammation can damage healthy tissue. Corticosteroids are often given for weeks if thrombocytopenia (low platelet count) is present without hemolytic anemia and for months is hemolytic anemia is also present. In addition, intravenous immune globulin (IVIG) may also be administered for patients. IVIG involves the administration of a blood product containing immune proteins (antibodies) obtained from many donors. The use of IVIG is less common if only hemolytic anemia is present without thrombocytopenia. 

If the individual does not respond to corticosteroids and IVIG or in case of relapse after treatment, administration of rituximab may be considered. Rituximab is a drug that is used to treat certain autoimmune conditions and cancers by slowing the growth of particular groups of immune cells. Although some people do not respond to rituximab at all, others experience long periods of remission following treatment. Rituximab is particularly effective in the case of Evans syndrome that is secondary to systemic lupus erythematosus or a tumor. 

Finally, in some cases, a splenectomy or the surgical removal of the spleen might be considered. However, splenectomy is becoming less common as a treatment option for Evans syndrome due to a low response rate, higher possibility of relapse, and the chance of complications. A splenectomy could lead to immediate improvement but relapses are common and occur within a few months after the procedure. In some individuals, a splenectomy may result in remission for long periods of time. 

 

The outlook of Evans syndrome varies among individuals. Some may experience long periods of remission when their symptoms disappear or improve over time. The effectiveness of treatment is also variable. Others may not have any remissions. Individuals affected by Evans syndrome are more likely to develop other autoimmune conditions. In some cases, severe infections and bleeding (hemorrhage) may occur. Evans syndrome could potentially become fatal in some affected individuals.

Tips or Suggestions of Evans Syndrome has not been added yet.

Al Hazmi A, Winters ME. Evans Syndrome. Clin Pract Cases Emerg Med. 2019 Feb 26;3(2):128-131. doi: 10.5811/cpcem.2019.1.41028. PMID: 31061968; PMCID: PMC6497206.

 

Genetic and Rare Diseases Information Center. Evans syndrome. 2014. Available from https://rarediseases.info.nih.gov/diseases/6389/evans-syndrome#:~:text=Evans%20syndrome%20is%20a%20very,destruction%20of%20red%20blood%20cells%20).

 

Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018 Oct 10;9:171-184. doi: 10.2147/JBM.S176144. PMID: 30349415; PMCID: PMC6190623.

 

Norton, A. and Roberts, I. (2006), Management of Evans syndrome. British Journal of Haematology, 132: 125-137. doi:10.1111/j.1365-2141.2005.05809.x

 

Shaikh H, Mewawalla P. Evans Syndrome. [Updated 2020 Jun 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK519015/

 

Community Details Update Created by RareshareTeam
Last updated 19 Aug 2020, 01:31 AM

Posted by RareshareTeam
19 Aug 2020, 01:31 AM

Hi everyone,

The Evans Syndrome community details have been updated. We added more information about the cause, prevalence, symptoms, diagnosis, and treatment. Hopefully, you find it helpful. 

News article on Evan's Syndrome added to Disorder Resources section. Created by RareshareTeam
Last updated 23 Feb 2020, 09:57 PM

Posted by RareshareTeam
23 Feb 2020, 09:57 PM

See Disorder Resources or RareShare external newslinks.

Community External News Link
Title Date Link
Evans syndrome: Everything you need to know 02/23/2020
Community Resources
Title Description Date Link

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

 

Expert Questions

Ask a question

Community User List

Pyoderma gangrenosum 1994-97, ending with ITP.

 

Evans Syndrome 2012 with all three counts (red, white and platelets) way low. Steroids, chemo and transfusion seem to have me back on track...
I have been diagnosed with Evans Syndrome three months ago. Prior to that, I had worked,with my hemotologist to manage ITP for three years ....
Have had many bouts of auto immune disease...the last being simultaneous Warm and Cold Aglutanin Hemolytic Anamia. Treated with High Dose Cytoxan 14 months ago. Also have idiopathic thrombocytopena...
Dx CLL April 2005; FCR completed12/2006; Campath consolidation completed 12/2007. CLL complete remission. Dx Evan's syndrome 10/2008.

Start a Community


Don't See Your Condition On Rareshare?

Start your own! With a worldwide network of 8,000 users, you won't be the only member of your community for long.

FAQ


Have questions about rareshare?

Visit our Frequently Asked Questions page to find the answers to some of the most commonly asked questions.

Discussion Forum

Community Details Update

Created by RareshareTeam | Last updated 19 Aug 2020, 01:31 AM


Communities

Our Communities

Join Rareshare to meet other people that have been touched by rare diseases. Learn, engage, and grow with our communities.

FIND YOUR COMMUNITY
Physicians

Our Resources

Our rare disease resources include e-books and podcasts

VIEW OUR EBOOKS

LISTEN TO OUR PODCASTS

VIEW OUR GUIDES

Leaders

Our Community Leaders

Community leaders are active users that have been touched by the rare disease that they are a part of. Not only are they there to help facilitate conversations and provide new information that is relevant for the group, but they are there for you and to let you know you have a support system on Rareshare.