Evans Syndrome in a Nutshell

by C. McNamara Romanowski
DISCLAIMER: This document is being written by a layperson who has Evans Syndrome, not a healthcare professional. The information presented herein is provided with the understanding that it is not "gospel", as it were, but a frank look at Evans Syndrome facts and statistics from a layperson's point of view. This information should never replace advice given to you by your doctor or other healthcare professional.  
Evans Syndrome is an uncommon condition defined by the combination (either concurrently or sequentially) of an attack on two or more lines of blood, including red blood cells (Autoimmune Hemolytic anemia), platelets (Ideopathic Thrombocytopenia or ITP), and white blood cells, also known as leukocytes. This occurs when the immune system mistakenly targets these cells for destruction, and the spleen subsequently destroys them. Autoimmune cytopenia can be a sign of systemic autoimmune disease, such as Lupus or other connective tissue disorder, and the patient should be tested appropriately. (Mikhail Shtivelband, MD, PhD, and http://www.ncbi.nlm.nih.gov/pubmed/16398647)

A common misconception that we see in the general public seems to be: "If it's not cancer, then it must not be serious." It's true that Evans Syndrome is not cancer: It is not true that Evans Syndrome is not serious. Evans Syndrome is very serious. A report in the Journal of Pediatric Hematology/Oncology showed that Evans Syndrome has an approximately 7% mortality rate after 3 years. (http://journals.lww.com/jpho-online/pages/articleviewer.aspx?year=1997&issue=09000&article=00005&type=abstract) This study was published in 1997, and treatment has changed since then, so the mortality rate may not be quite as high today; however it is the only solid statistic with regard to mortality that can be provided in the text you are currently reading.

In order to obtain a diagnosis of Evans Syndrome (ES), certain other health problems must first be eliminated. For example, the symptoms of ES are very similar in nature to leukemia and lymphoma. As a result, both leukemia and lymphoma must be ruled out prior to a diagnosis of ES. A bone marrow biopsy is done to closely examine the body's mechanism for blood cell creation. A battery of blood tests reveals that there is no other reason for the low blood counts, as well as the presence of antibodies that are targeting healthy blood cells for destruction (Coombs test).

While awaiting diagnosis, blood transfusions may be given, as well as Intravenous Immunoglobulin (IVIg), which coats the blood cells, protecting them from destruction; and IV steroids, which slows down the immune system, helping to shut down the mechanism that is destroying the blood cells.

Once a diagnosis of Evans Syndrome is obtained, aggressive therapy is undertaken in order to shut down the immune system reaction to the blood cells. Most frequently this is done with very large doses of corticosteroids such as Prednisone or Decadron. Evans Syndrome does tend to be refractory to treatment, in which case additional medications may be used, including but not limited to certain chimeric monoclonal antibody treatments (Remicade, Rituxan, etc.), and anti-rejection drugs (Imuran, Cellcept, Cyclosporine, etc.). 

If a person with ES still does not respond to treatment, or should they continue to experience refractory episodes, splenectomy (removal of the spleen) may be considered. This is an invasive procedure that leaves the person without all of the body's natural defenses to infection. Because of this, a person without a spleen is wise to take extra precautions to prevent infection or exposure to illness throughout the rest of their life. Unfortunately, splenectomy is not always successful, sometimes requiring further treatment with the aforementioned drugs even after removal of the spleen. 

There are cases where no amount of drug treatment or surgical intervention helps, and the doctors may then recommend a bone marrow transplant. This is a very dangerous procedure, as the person must be given a battery of drugs to kill off their immune system, and then receive bone marrow stem cells from a donor. Marrow transplants for cancer can be done with autologous cells (taken from the person who is also to receive the transplant), however it is the understanding of this author that this is not the best choice for a person with Evans Syndrome.

Evans Syndrome has no cure. Today, the best that can be hoped for is lifetime remission. ES is considered to be an "orphaned" disease, as almost no research has been done. The reason for this is that there are so few people with ES that it is not fiscally responsible for drug companies to do research into a treatment or cure.

We are the Evans Syndrome Community Network, a non-profit organization serving those who are affected by ES. Our mission is to provide a safe place for networking between individuals whose lives have been impacted by Evans Syndrome (ES); to make available scientific and educational material, online and in medical environments, fostering learning about ES among our Community and the general public. We will assist families affected by ES with travel, lodging, and medical expenses; collaborate with other organizations that share our vision, and fund autoimmune research to benefit our international Community.

Should you need to contact us directly, for any reason, please drop us an email.
Thank you for asking about Evans Syndrome. Together we will make a difference, standing shoulder to shoulder.
Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.

What is this thing called Evans Syndrome?

by C. McNamara Romanowski

Evans Syndrome...Most people have never heard of it. Most health practitioners don't even know what it is.

Evans Syndrome is a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenia. What does this mean? It certainly doesn't mean you need to take iron. It doesn't mean you need a "cleanse". It's not something that can be cured with magnets or homeopathy.

Evans Syndrome may rear its ugly head at any time during a person's life. The mean, or average age of diagnosis of Evans Syndrome is 7.7 years.

Evans Syndrome is a perfect storm, when the body's own immune system targets the red blood cells and platelets as organisms foreign to the body, and then the spleen destroys them. People with active Evans Syndrome have trouble clotting blood, causing a tendency to bruise and bleed. The skin on the hands and arms, feet and legs, and torso may reveal tiny, pinprick-sized red or purple dots called petechia, which are formed by little hemorrhages under the skin.

Another thing that happens is destruction of the hemoglobin, the protein in red blood cells that gives them the red color. It is this part of the cell that carries oxygen to the rest of the body. A person with active Evans Syndrome may experience tissue breakdown or even congestive heart failure.

After reading the above paragraphs, you may wonder why the spleen isn't just removed, thereby taking away the destructive factor. Well, the body is an amazing and wonderful thing, and it can often find alternative means of protecting itself. Hence, removal of the spleen is only permanently effective in 30-70% of cases (depending upon which study is examined); and then the patient is left without one of the body's main defenses. Our spleen is not like our appendix. We actually need the spleen: It is used to fight infection in the body.

Usual course of treatment to combat Evans Syndrome includes intravenous immunoglobulin (IVIG) and a long course of very strong steroids. IVIG is only a short-term solution because it is extremely expensive and it does not produce remission. IVIG works by making blood cells slippery, and, in a way, fooling the immune system into ignoring them. IVIG is very expensive expensive because it is a blood by-product that requires the donations of anywhere from 5,000 to 30,000 people.

At some point in most the lives of most people, small doses of steroids are given to treat things like allergies or back pain. Steroids are superb anti-inflammatories. There is a problem, though: Steroids are what may be called a "sweet poison". The benefits, especially of long-term treatment, must be weighed with the detriments. Steroids are immediately addictive. Not only that, but they can cause a host of other problems including, but not limited to: Cushing's syndrome; diabetes; osteoporosis; cataracts; glaucoma; kidney failure; thinning of the skin; morbid weight gain; and immunodeficiency. This last side-effect is why steroids are useful in the treatment of Evans Syndrome: They effectively shut down the mechanism that is being used by the body to attack the blood cells. Eventually, though, the steroids must be tapered and then stopped. At this point the body either begins the attack anew, or the person goes into remission.

The next line of treatment can involve the use of any number of drugs, including full-blown IV chemotherapy. Sometimes chemo drugs such as imuran may be helpful, and there are a couple of newer drugs that encourage the production of platelets, also serving as a deterrent. That said, however, there is no real "magic bullet".

Many hematologists turn to Rituxan, a sort-of genetic therapy which is manufactured from the ovaries of genetically-altered Chinese hamsters. Who thought of this? I have absolutely no idea. Rituxan was first used to treat leukemia, and later lymphoma. Since the symptoms of these cancers are similar to the symptoms of Evans Syndrome, it was decided to try the treatment on a patient with Evans. Because of its effectiveness in treating Evans, Rituxan has also been used to treat other autoimmune illnesses, such as Lupus SLE and rheumatoid arthritis.

Rituxan is usually given in 4 doses over a 5-week period. There are almost no side effects except those which may occur during infusion: Because of this, precautions for anaphylactic shock should be taken while the treatment is being administered. Rituixn has about a 70% rate of response in children, and about 50% in adults. Results may start to be seen anywhere from 2-6 weeks after the last infusion. If Rituxan doesn't work, there is no sense in trying it again...But if it does, it is should be given consideration should the person relapse.

If all of these drugs are tried, and still the patient is not responsive, removal of the spleen (splenectomy) is probably indicated. Some medical professionals use this as the first course of treatment, however, it is felt in other circles that non-invasive treatments should be tried prior to invasive treatments like surgery.

The final course of treatment available to people with Evans Syndrome is a bone marrow transplant. This is the last choice because of both pre and post-transplant complications which may occur. First, the person must undergo a scourge of drug therapies in order to kill the bone marrow and shut down the immune response. Next, new cells are introduced, sort of "resetting" the body's marrow-producing capabilities. Sometimes these cells can be gleaned from the patient's own marrow: Other times donor cells are used.

If, at any time, the body stops attacking the blood cells, this is referred to as "remission". There is no real cure for Evans Syndrome.

How do I know this? I am not a doctor or a nurse. I do not work in a lab, and I don't have a degree in biology. I was diagnosed with Evans Syndrome at the age of 44. I have been in remission for 5 years, following a 5-week round of Rituxan. I first started showing signs of a problem around the age of 2.

Sometimes I feel like there's a dark cloud following me around, waiting to once again unleash that "perfect storm". But for the must part, I am just grateful to be alive.

For further information and support, register with the Evans Syndrome Community Network on Facebook, or with the Evans Syndrome Group on Yahoo.

Copyright © 2012 by Evans Syndrome Community Network. All rights reserved.