Splenomegaly (enlarged spleen) is common in thalassemia major and intermedia due to the high rate of hemolysis (red blood cell destruction). This takes place because the spleen sees the defective red cells of the thalassemic as deficient and the transfused red cells as invaders (much the same as with host vs graft disease) and removes them from circulation. One should note that although natural made normal red cells live for about 120 days, transfused cells last a matter of weeks, at the most. Antibody reactions can also exacerbate this problem. This hyperactivity of the spleen results in splenomegaly due to the amount of blood cells that are filtered out and also by a physical increase in size of the spleen so it can handle what it sees as a heavy load of blood cells that need to be filtered out. What has been observed by us in thalassemia minors, apparently has not been recorded by the medical establishment which persists in the notion that thalassemia trait has no symptoms. This actually defies logic, as a great number of minors would be predicted to have health issues by the very nature of their own blood. While beta + minors may see few if any symptoms, beta - or 0 minors will have a less than adequate hemoglobin level because one of their hemoglobin genes is producing little, if any beta globin, which does result in lower hemoglobin levels and also a surplus of alpha globin, which can also add to the problems by adding "clutter" to the bloodstream. As has been noted, splenomegaly is more common in pregnant minors and in some cases can present a great danger, as it did with Courtenay. However, splenomegaly is not confined to pregnant women among thal minors. Many minors report this, even if the doctors have chosen to ignore the pattern. And of course, we know we have heard from hundreds of thal minors in this group who report other health issues, in addition to enlarged spleen. Thalassemia minor does cause problems and it is not rare, as the literature tries to portray it. This is very frustrating for patients who do have serious quality of life issues because of thal minor but can't get any doctor to admit that there is a connection.
For many years, the response to splenomegaly was splenectomy. In recent years, this has begun to change, as patients who have had their spleen removed are observed over many years, and the problems created by the splenectomy become more apparent. Chief among these is the tendency towards clotting issues which can lead to stroke. New therapies are needed to counter the hyperactive spleen but as of yet, little if anything has been suggested to patients. So, I am going to go out on a limb and make some suggestions. All thalassemics, minor through major should be on a program of supplementation and one goal of this should be to create a healthier circulatory system with healthier red blood cells. Folic acid, natural vitamin E, IP6 (phytic acid), vitamin D and magnesium are all necessary for a healthier circulatory system. Healthier red cells are less likely to be destroyed by the spleen and efforts to improve their health should be taken. I think some emphasis needs to be put on magnesium. A recent study showed that patients with higher levels of magnesium had fewer gallstones. See
http://www.thalassemiapatientsandfriends.com/index.php?topic=1593.msg13119#msg13119Researchers may not be aware why this happens but I have a theory. Magnesium improves the health of the walls of red blood cells. One of the main goals of the spleen is removing the weak red cells and the strength of the cellular wall is a factor in whether or not red cells are filtered by the spleen. The more red cells that are destroyed, the higher the amount of bilirubin which leads to gallstones. While researchers may want to focus on one organ as though it exists in a vacuum, in reality, the body is a complex organism with all aspects interconnected. A researcher may be able to separate the gallbladder for the purpose of a study, but our bodies can't and don't. It is all interconnected. I do believe that the patient can take some actions to counter the hyperactivity of the spleen and that this has to begin with providing the over stressed body with sufficient nutrients to counter these stresses and to reduce the amount of red blood cells being filtered by the spleen.
As patients, are you willing to be dismissed by your doctors as hypochondriacs and complainers or are you going to be willing to be proactive and take the steps necessary to optimize your health? Since the doctors are almost universally unwilling to address the health issues of thal minors, it leaves the minors with little choice but to take action on their own and do what they can to improve their health.