While stroke is more common in Alpha Thalassemia and sickle cell anemia, it is also a danger in thal major and intermedia. Keep in mind that some percentage of thals will have strokes similar to the percentage in the general population.
From
http://moon.ouhsc.edu/kfung/jty1/neurohelp/ZNB0DD02.htmBeta-thalassemia major... have an increased risk of thrombotic stroke, to which the postsplenectomy thrombocytosis contributes.
It had been found that there is a chronic hypercoagulable state in thalassemia. What this means is that the blood sticks together more than it should, creating a higher possibility of blood clots, which can lead to strokes and heart attacks. It has been found that the risk is higher among non-transfused intermedias and majors who do not receive adequate transfusions and also among splenectomized patients. The reason for this is suspected to be a combination of having more damaged red cells in the blood stream because of the body's own red cell production which is elevated in untransfused patients, and also increased platelet counts. Thalassemic patients also have low levels coagulation inhibitors, protein C and protein S.
This research can be seen at
http://www.bloodjournal.org/cgi/content/full/99/1/36A range of laboratory tests has provided solid evidence for the existence of a chronic hypercoagulable state in thalassemia and, particularly, in splenectomized patients with beta -TI who do receive regular transfusions...(intermedia) patients may be more susceptible to thromboembolism because they have more circulating damaged RBCs and increased platelet counts. The beneficial role of regular blood transfusions is illustrated by the observation that thromboembolic (editor: blood clotting) manifestations are more frequently recorded in less developed countries with limited transfusion resources and ex vivo and in vitro experiments that show that normal RBCs can eliminate the abnormal aggregation observed with thalassemic RBCs.
It is suggested that a therapy including blood thinners or anti-coagulant agents may be of help in patients who have had previous thrombotic events (clots) or who may have a tendency for them.
Many thal pateints have used blood thinners because of high platelet counts and the danger of clots that this presents. Vitamin E and aspirin are also well known as blood thinners that may provide some protection against clotting. In fact it is demanded that both be stopped one week before surgery because of their anti coagulating properties, and of course, after surgery, blood must be able to clot normally to prevent bleeding. This property is one more benefit vitamin E offers for thals.
What does all this mean to you? You do have an increased chance of blood clots, and therefore strokes which are caused by clots, if you are thalasssemic, but even more so in non-transfused patients who've had splenectomies.
If you have not already seen a doctor about what happened, do so as soon as possible. Silent strokes do occur in patients and can be a precursor of a more serious stroke. You need to be examined immediately and find out if what you experienced has anything to do with stroke. These same symptoms can also be a result of a drop in blood pressure and may only be a temporary situation, possibily related to low Hb. However, it is very important for
ALL thalassemics to watch for
ANY stroke like symptoms and report them to your doctor immediately. It may be nothing but because of the serious nature of strokes it should never be taken lightly. I don't want to scare anyone and want to emphasize that
in most cases this is simply a precaution to make sure that nothing serious has occurred. The research mentioned in this post also directly relates to an earlier post which I made that stated that thrombotic events were observed to be more common in splenectomixed patients.
http://www.thalassemiapatientsandfriends.com/index.php?topic=297.15Recent observations and case reports have shown that splenectomy in such patients may cause serious venous thrombotic events ranging from deep vein thrombosis to pulmonary thrombotic lesions complicated by pulmonary hypertension.
Also from
http://www.bloodjournal.org/cgi/content/full/99/1/36Cappellini et al observed a high incidence of venous thromboembolic events (VTEs) in a group of 83 patients with beta -TI who were followed for 10 years. Twenty-four patients (29%) developed either pulmonary embolism, DVT, or portal vein thrombosis, and recurrent VTEs occurred in 9 of these cases. All patients except one had undergone splenectomy.
Autopsy findings in patients with thalassemia have clearly demonstrated hypercoagulability as a pathologic feature. Autopsies on 17 splenectomized and 2 nonsplenectomized patients of 43 with beta thalassemia/hemoglobin E disease showed atherosclerotic changes and obstructive lesions consisting of organized, recanalized thrombi in the pulmonary arteries and microvasculature...The results from these studies show that, in addition to their increased number in splenectomized patients, chronic platelet activation is present in beta -TM and beta -TI...The presence of morphologic platelet abnormalities in splenectomized patients with beta thalassemia/hemoglobin E disease may also contribute to an enhanced risk of vascular complications
This is found far more often is splectomized patients who do not transfuse.
It is now being recognized that there is a second danger to the heart in thalassemics in addition to iron overload. As thal patients age, this is becoming more prevalent. The incidence of thrombotic events is very likely related to this hypercoagulable state and therapy including anti coagulants and blood thinners may become the answer.