Today we will be discussing transfusion complications in thalassemia patients. Thalassemia is a group of genetic blood disorders with a defect in the globin polypeptide chain of hemoglobin that results in anemia. Red blood cell transfusions are the primary treatment for thalassemia patients.
The United States Centers for Disease Control and Prevention evaluated the transfusion complications in thalassemia patients. Dr. Vichinsky led the study and noted:
“There are probably about 350,000 births a year in the world with babies with hemoglobinopathies. And historically only a few of them migrated to the United States. In the last 30 years there’s been a dramatic change in immigration patterns in the United States that reflect regions that have a very high rate of thalassemia mutations.”
The study enrolled more than 400 thalassemia patients over a nine year period, who were predominantly Asian and Caucasian. They found that 80% of their patients received eight or more transfusions per year. Over three quarters of the patients received treatment for hemosiderosis. Iron overload commonly induced organ dysfunctions including cardiac disease, gonadal failure, growth hormone deficiency, and other complications.
Almost a quarter of transfused patients had also been exposed to an infectious disease, including hepatitis A, B and C, parvovirus, HIV, and malaria.
In addition, allergic, febrile or hemolytic transfusion reactions occurred in almost half of all patients, and almost 20% of all transfused patients developed alloantibodies.
Dr. Ambruso wrote an accompanying editorial and notes:
“We have to consider more extended matching and we have to consider matching not only for ABO and Rh but we have to look at C, D, and E and perhaps we have to look at other minor group antigens as well.”
Currently, there are not standardized guidelines for red blood cell antigen matching for thalassemia patients. Dr. Vichinsky also pointed out that:
“What this data shows me is more work is needed, blood banks need to come up with prospective transfusion protocols that can safely monitor the population and determine the cost efficacy of genotype phenotype screening for patients and should really begin to look at this issue of infections.”
We’ll be back with another edition of Transfusion News on April 30. Thanks for joining us.
References
1. Ambruso D. Transfusion Complications in Thalassemias. Transfusion 2014;54(957-959).
Transfusion offers CME credit for this study! Log on at www.wileyhealthlearning.com/trf
I would kindly like to request you to explain why there was Iron overload in 80% of patients who received 8 or more transfusion a year and yet this has been a gradual transfusion process over such a long period of time.
Then I thought most of the units of blood issued to patients are crossmatched for compatibility and also specific unexpected antibodies are Identified.if patients blood phenotypes are determined what will happen to individuals who type with the negative antigens, since that would be the problem as regards to further transfusions ie C, D,E,K or jka. Note for sickle cell there is a provision to issue (C,E and K)negative red blood cells.
Iron overload is a common problem among individuals who are chronically transfused since there is approximately 1 mg of elemental iron in every 1 mL of transfused RBCs. Thus, the findings by Vichinsky et al. that 75% of individuals who have been chronically transfused and consistently received eight or more units of RBCs is expected.
The majority of thalassemia patients are transfused crossmatch compatible RBCs, but often do not receive phenotypically matched RBCs for C, E, and K1. Vichinsky et al. found that 19% of thalassemia patients became alloimmunized. Thus, Dr. Vichinsky suggested that thalassemia patients, often similar to sickle cell patients, should receive more extensively matched RBCs than just ABO and D.