EVANS SYNDROME


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WHAT IS EVANS SYNDROME?


     Evans syndrome is a rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. Patients are diagnosed with thrombocytopenia and Coombs' positive hemolytic anemia and have no other known underlying etiology. The patients may be affected by low levels of all three types of blood cells at one time, or may only have problems with one or two of them. The specific cause for Evans syndrome is unknown and it has been speculated that for every case, the cause may be different. There have been no genetic links identified.
     The course of Evans syndrome varies by case. The patient may be symptomatic of whatever blood levels are down. If the red blood cells are down, the problems complained of may be weakness, fatigue, shortness of breath and the usual things associated with anemia. With low platelets, they are susceptible to bleeding and major bruising from minor bumps and cuts. A bump on the head could cause severe brain hemorrhage and death. With low white blood cells, the patient has increased susceptibility to infections and difficulty in fighting these infections. The patient may have problems with one, two or all three of these blood lines, at one time.
     Treatment of Evans syndrome varies and there has been no "magic bullet" identified that will cure this. Steroids are frequently used to help suppress the immune system, or to decrease the production of the "bad antibodies". Intravenous immune globulin or IVIG is often tried as is chemotherapy when responses to other treatments are not satisfactory. Splenectomy has frequently been done, but the benefits of this are usually short-lived. In the last study done, the beneficial effects from splenectomy had only lasted an average of one month. Closely monitoring the patients' complete blood count is crucial to the patients' treatment. Transfusions, of blood products, is done in crisis situations to help stabilize the patient but is not a long lasting solution as these cells are usually destroyed very quickly by the body.
     The prognosis with Evans syndrome is guarded. Some patients have episodes of major blood cell destruction followed by long remissions, while others have chronic problems with no remissions. It has been reported that patients with Evans syndrome have a greater tendency to develop other autoimmune disorders such as lupus and rheumatoid arthritis and there is a tendency to develop various malignancies. Careful monitoring of the patient by a qualified physician is very important.


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A QUICK LESSON IN BLOOD COUNTS...


     Red blood cells or erythrocytes comprise a third of all the cells in the human body. With about 20 trillion in the average adult, they're not only the most common cells in the human body, they are also the only cells that don't have a nuclei. The red blood cells' primary function is to carry oxygen to the body's tissues, and because they are 40 percent lighter than they would be with nuclei, it is easier for the heart to pump them through the circulatory system.
     Red cells contain a molecule called hemoglobin, which picks up oxygen as the cells travel through the blood vessels in the lungs. After red cells leave the lungs they get distributed throughout the body, passing through ever-smaller vessels called arterioles and capillaries. There they get forced up against the vessel walls like passengers in a crowded subway train. Under that pressure, the oxygen molecules pop off the hemoglobin and get taken into the vessel walls and passed on to neighboring cells. Relieved of their burden, the red cells pick up carbon dioxide, which they then carry back through the veins to the lungs to be exchanged for new loads of oxygen.
     White blood cells or leukocytes, are the body's border defense: they are responsible for apprehending and eliminating foreign substances. They're larger but much less numerous than red blood cells, numbering about 20 million in the adult body. While red cells are buoyed along like cargo rafts as the heart pumps the serum, white cells function more like patrol boats. Under their own locomotion, they can change direction to go after an invading molecule or cell.
     There are three basic types of white cells-granulocytes, monocytes and lymphocytes-each with its own specialized tasks. Granulocytes, the first line of defense, home in on bacteria or any other foreign substance in the blood. They are filled with granules containing chemicals that destroy their prey. One type of granulocyte, the neutrophil, is an all-purpose search-and-destroy agent that makes up 60 percent of white blood cells. The other two granulocytes, eosinophils and basophils apprehend special intruders such as allergens and parasites and account for 3 percent of the white blood cell population.
     About 33 percent of white cells are lymphocytes, the brains of the immune system. They send out antibodies to immobilize foreign molecules and chemical messengers that spur other cells into action. The remaining 4 percent of white blood cells are monocytes, which clean up cellular debris and devour invaders that have been tagged with antibodies. (Like what happens to the red blood cells, platelets and sometimes white blood cells themselves in Evans syndrome when they have been tagged with antibodies.)
     Platelets, so named because they are shaped like tiny plates, release chemicals that promote clotting when blood vessels are broken. These chemicals work by assembling a blood protein called fibrin into a meshwork over the damaged area. The mesh serves as a net to trap blood cells and proteins, which eventually form a plug in the damaged vessel wall.
     The meaning of the numbers: A complete blood count involves computing the number of red cells, the percentage of red cells in the whole blood, the size of the average red cell (an indication of its robustness), the average amount of hemoglobin per red cell, and how much there is in the blood. In addition, a physician will want to calculate the total number of white cells and the percentages of the different types of white blood cells, which can be distinguished under a microscope by the shapes of their nuclei. A physician will compare the numbers of cells in a sample of a patient's blood, with the average number of cells in the same amount of blood from a healthy person. Having too few red blood cells or too little hemoglobin is called anemia. Having too few white blood cells is a sign that a person's immune system is not functioning properly. Too many white blood cells usually means that the patient has an infection. The type of white cell that is over produced can indicate the extent of infection. An increase in the number of neutrophils, for example, means the person probably has a bacterial infection. An increase in lymphocytes and basophils usually corresponds to an allergic reaction like what you might get from ragweed. If there are far too many white blood cells-in the neighborhood of 100,000-the patient may have leukemia. A physician will also look at the number of platelets in the specimen to assess if the patient may have too few or too many.
     While normal values vary slightly from institution to institution, the following values are within the accepted range of normal:

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PROFILE OF A CHILD WITH EVANS SYNDROME


     Katie was diagnosed with Evans syndrome when she was 13 months old. For the five months prior to her diagnosis she had been noted to have a lot of bruising of her arms and legs. They were just small bruises most of the time and because she was getting older and was crawling and learning to pull up, they were attributed, by her doctor, to bumping herself. During those same five months Katie had a low grade fever every day. Her temperature rose to 99.6 to 100.6 every evening. She was cutting teeth, so it was assumed that that was the cause of her fevers. She had one virus and stomach flu after another with rarely ten days of good health before being struck by something else. It was odd because Katie didn't attend day care and wasn't out in public very much so it was hard to imagine where she was being exposed to all of these "bugs". Both my husband and myself were lucky enough to be able to stay home with her full time at that time, as we had adopted Katie after ten years of adoption attempts, and she was the center of our universe. We weren't bringing "bugs" home from work.
     On her "well baby check up" at 12 months, I had again complained to her doctor that something did not seem right with her. My concerns were passed off as "normal baby problems and nothing to be concerned about." We returned to the doctor's one month later and I insisted that blood work be done at the hospital, as I knew something was very wrong. Despite a normal assessment by the pediatrician, he wrote orders for the blood work and we headed for the hospital with the understanding that he would call me later that day with the results.
     Once home from having the blood work done, Katie was crawling on our living room rug and bumped her front tooth. She started to bleed and although the bump had only been minor, we could not get the bleeding to stop. She continued to ooze for an hour when I called the doctor to report my concerns and ask about the lab results.
     A few calls later and the doctor was on the line telling me that he thought she had leukemia and that the pathologist who had reviewed the blood work thought that she had some type of bone marrow tumors. Her platelets, red cells and white cells were all so very low that she was rushed to the local hospital for stabilization and then transferred to the university hospital for further evaluation and treatment.
     After bone marrow biopsy results showed normal but increased activity, the cancers were ruled out and the diagnosis of Evans syndrome was made. Katie has been treated with steroids both IV in crisis and by mouth routinely since. She used to spend almost a week per month in the hospital with infections, sepsis or crisis status blood levels. After three and a half years of that, I started begging her doctors to try something different. I am a registered nurse and had by then spent a lot of time investigating Evans syndrome and the best ways to treat it. I was convinced that if we tried giving her the immune globulin (IVIG) routinely instead of waiting until her levels crashed, that we might make some progress. Finally in the spring of 1995, they agreed to let me try my theory.
      Katie had an infusaport placed which is a type of permanent IV line that goes into a big vessel in the body and is under the skin so normal activities like swimming don't affect it. I started giving her the IVIG at home every three weeks, sometimes every two when her levels would drop more or if she started to get sick. For Katie this was the magic ticket. Until 1998, Katie did not require any hospital stays for infections or crisis levels. That' s not to say that she hasn't been sick, but we've been able to manage her at home. She still has blood work done every week to see how she's doing. Katie is now eight years old. She is in home school became she has poor endurance and has too many problems with low white blood cell counts to be able to fight the normal childhood illnesses she would be exposed to in school. She has a few friends that she loves to see and spends many hours each day playing on her computer. She loves to be out in public with people, and she does get out, but we try to keep her out of anywhere crowded and away from anyone sick. Very tough during flu season!Katie's Evans syndrome was triggered by her DPT immunizations when she was an infant. She had violent reactions to the three rounds that she got, and began with the symptoms (bruising and fevers) of her Evans syndrome within a week of her last immunization. She also had a grand mal seizure and respiratory arrest six hours after her last immunization. When she was diagnosed, I was told that I would never find another child with Evans syndrome and unfortunately, I have found more than 25. Another little boy diagnosed with Evans syndrome around the same time as Katie, I discovered received the very same lot number of DPT vaccine that Katie got!I have done hours and hours of research on this subject, and have found documentation in all of the hematology and immunology textbooks, that the DPT vaccine and some of it's preservatives and additives that make it work better (thimerosal and aluminum salts), have all been found to be related to thrombocytopenia and hemolytic anemia. I do not mention this for the purpose of upsetting anyone, or to convince anyone that their case of Evans syndrome is also related to vaccines, because it may or may not be. It is merely food for thought.

Lou Addington, mother


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EVANS SYNDROME RESEARCH AND SUPPORT GROUP


     The Evans Syndrome Research and Support Group was founded in 1992 by Lou Addington and Sandra Mathews. The purpose for the group was to try to locate as many cases of Evans syndrome as possible, to gather more information about the disease and to offer support to other families facing the heartache of this devastating illness. Our main focus has been Evans syndrome in children because the diagnosis of Evans syndrome in our daughters is what touched our lives and sparked this fire. However, we welcome anyone with this syndrome to register with our group. We can all benefit from each other through a sharing of information and support.
     We were instrumental in inspiring Dr. Wang at St. Judes hospital to do a national survey to attempt to identify the incidence and management of Evans syndrome. We mailed out his survey to all of the hematology/oncology physicians in this country and the results were sent to Dr. Wang and his team who collated and processed the information that was later published in his paper in the Journal of Pediatric Hematology/Oncology in 1997.
     I believe that the incidence of Evans syndrome is much greater than has been generally believed by the health professionals. I have received calls from many distant places including Spain, Scotland, Canada, South America and Brazil! It does not seem to me now, to be the "rare disease" I once thought it was. We need to try to gather more information so that perhaps we can get more answers. On this web site is a questionnaire (quite lengthy, I'm sorry!). If you or a loved one has been diagnosed with Evans syndrome, please complete it and return it to us by FAX or the post office. We will compile the information and look for patterns and trends that we can then call to "someone's" attention.


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