Discussion Forums > Thalassemia Major
can someone help me out?
Andy Battaglia:
Hi Sharmin,
I would suggest 1 mg folic acid daily. Adult thals are known to take 5-10 mg daily. 100-200 iu Natural complex vitamin E daily.
Although the autoantibody response is not directly related to thalassemia, it may be related to the transfusions. It has become apparent in recent years that the incidence of this in transfusing thals has been underestimated because of lack of data on the subject. As more data has been collected, the relationship has become more apparent.
http://asheducationbook.hematologylibrary.org/cgi/content/full/2006/1/13#R8
--- Quote ---Association with blood transfusion
While alloantibody formation is a recognized and reasonably common complication of blood transfusion, the possibility of autoantibody formation has not been well recognized. Young et al analyzed over 2600 patients with a positive direct antiglobulin test or indirect antiglobulin test, and identified 41 patients who had both an autoanti-body and an alloantibody. About a third of them developed their autoantibody in close temporal association with alloimmunization following recent transfusion.7 Therefore, AIHA (Autoimmune hemolytic anemia) developed either concurrently or shortly after alloimmunization from blood transfusion. The authors conclude that AIHA is a potential complication of allogeneic red blood cell transfusions, and recommended supportive treatment with iron and erythropoietin analogues, avoiding further transfusion whenever possible. This complication of red blood cell transfusion may be more common than previously appreciated.8 It has also been noted in patients with hemoglobinopathies who have received multiple transfusions.9–11
--- End quote ---
A study from Oakland Children's Hospital published in 2000 investigated the immune response among those of Asian descent who received blood transfusions with the blood of predominantly white donors.
http://bloodjournal.hematologylibrary.org/cgi/content/full/96/10/3369
--- Quote ---Alloimmunization and autoimmunization are common, serious complications in Asian thalassemia patients, who are affected by donor-recipient RBC antigen mismatch and immunological factors.
World wide, thalassemia is most prevalent in Asians, and due to large population movements, people of Asian descent constitute the majority of patients in many thalassemia centers in Western countries. However, there is very limited data on the RBC phenotypes among Asians, the extent of alloimmunization, and the role of phenotype differences between blood donors and recipients in forming antibodies.
We studied the frequency of alloimmunization and erythrocyte autoimmunization among thalassemia patients who received regular transfusions and in particular looked at the RBC phenotypes of Asians. We investigated factors possibly affecting the antibody formation: RBC phenotypic differences between the local donor pool and Asian recipients; patient- and blood-related immune factors; and preliminary findings of conformational changes of the transfused RBCs, which perhaps trigger antibody response.
The association of thalassemia and erythrocyte autoantibodies has not been studied. The true frequency and the clinical spectrum are unknown.1 We found a high frequency (25%) of autoantibody formation, mostly IgG warm antibodies, of which 18% had a significant clinical hemolysis. The antibody development was associated with alloimmunization, exposure to nonleukoreduced blood, and absence of spleen
Our data show altered RBC deformability profiles, more so in patients without spleen than in patients with spleen (Figure 1). These findings are consistent with senescent erythrocytes and may expose new antigens and promote or enhance an immune reaction, as known to occur in aging and impaired RBCs.26,27 It is likely that the absence of an efficient filtering system for removal of damaged erythrocytes enhances the process. In accordance with this hypothesis, a previous study showed increased hemolytic autoantibody response in mice without spleen compared to mice with spleen and a spleen role in regulating this response.28
In summary, our data show that alloimmunization to minor erythrocyte antigens and erythrocyte autoimmunization, of variable clinical significance, are frequent findings in transfused thalassemia patients. The causes are not fully understood; however, data suggest that the recipient's immune status, along with the effects of multiple allogeneic blood transfusions, can induce antibody formation. We found an association with the absence of spleen and the presence of deformed ex-vivo RBCs that probably augments these mechanisms. The difference in the RBC antigen profile between the predominantly white donor population and the Asian recipients likely further contributes to the phenomena.
With the growing knowledge base of the immune effects of current blood transfusions23,24 and limited data on the immune status of thalassemia patients, a large study addressing the complex interaction of these factors is needed. Such information may enable understanding and prevention of this serious and common complication. In clinical and transfusion practice, when considering splenectomy, a potential higher risk for future RBC immunization should also be taken into account. We recommend obtaining a RBC antigen phenotype on all thalassemia patients, particularly of Asian ethnicity, who are started on transfusions, and if feasible, providing leukodepleted blood matched for antigens of the ABO-D and Kell systems. Recruitment of Asian blood bank donors, just like recruitment of black donors for SCD patients, can increase the availability of compatible blood for thalassemia patients, who have a life-long transfusion-dependent disease.
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An important note from this discussion is that splenectomy may actually make the autoantibody problem more likely and worse.
Although your son's transfusion requirements increased, they are not in any way, out of control. I agree that this will probably pass with prednisone use and that a temporary higher dose may clear it up. I think it would be preferable to knock it out with a high dose and eventually withdraw the prednisone, than continue long term use of prednisone. There is a very high percentage of this reaction going into remission in patients using prednisone. If there is a later recurrence, prednisone therapy is resumed. A very positive note is the lack of any visible effects on your son's health. He looks really good in the pics. :happyyes
Sharmin:
Wow Andy,
thank you so much - I am absolutely blown away by all of your research. You are so resourceful!
I have learned more from your message than I have been able to learn over the past two years! This knowledge has the potential to change our approach to his treatment - once we discuss it with his doctor. His doctor is absolutely wonderful, once he learns all of this I am sure he will be happy to take this approach.
:thankyou
Sharmin
Andy Battaglia:
Sharmin,
This has been a real challenge and as you can see from the discussion, far more work needs to be done to study patients and these immune system responses. Dr Vichinsky has been at the forefront of putting this knowledge into practice with more specific blood matching and I wasn't surprised to see his name on this article. The doctors on the west coast have seen more of the new generation of Asian thals and are making every attempt to deal with the new problems that arise in treatment and awareness. I would suggest a consultation with Dr Vichinsky at some point, if at all possible.
Zaini:
Hi Sharmin,
I hope everything will become smooth and easy for you and your son,give my love to little mr handsome :biggrin
ZAINI.
Manal:
Andy
I thought that the benefits of folic acid is only restricted to the formation of the blood ( it prolongs its life span and increase the red blood cells count ) , so how is it going to have the same effect on the ''already made blood'' i.e transfused blood?
By the way Ahmad takes 5mg folic acid daily
Manal
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