title: Study of alloimmunization and autoimmunization in Iranian β-thalassemia major patients.
abstract: BACKGROUND
  Thalassemia is one of the most common monogenic disorders characterized by reduced production of globin chains. Although regular red blood cell (RBC) transfusion support is the main treatment for these patients, it may be associated with complications such as RBC alloimmunization.
  
  
  OBJECTIVE
  The study aimed to determine the incidence of alloimmunization and autoimmunization to RBC antigens in β-thalassemia major patients from Zanjan, Zanjan Province, Iran.
  
  
  METHODS
  A total of 49 β-thalassemia major patients comprising 24 females and 25 males (mean age: 18.59 ± 8.16 years; range: 2-40 years) from Northwest Iran were included in a cross-sectional study. Alloantibody screening and identification were done using 3-cell and 10-cell reagent red blood cells, respectively. Autoantibody detection was performed using direct Coomb's test.
  
  
  RESULTS
  The incidence of alloimmunization was 16.32% with 10 alloantibodies identified in 8 patients. The most common clinically significant alloantibody identified in alloimmunized patients was anti-Kell (K-antigen) (60%) followed by anti-Rhesus (Rh) (E, c-antigens). The rate of alloimmunization was significantly lower in patients transfused with leukoreduced RBCs compared with those transfused with nonleukoreduced RBCs (9.53% vs 57.14%, P = 0.001). There was no significant correlation between alloantibody formation and the age, gender, hemoglobin levels, number of transfused units, and splenectomy.
  
  
  CONCLUSIONS
  Transfusion of leukoreduced and phenotypically matched red blood cells for Kell (K) and Rh (E, c) antigens may help reduce the alloimmunization rate in Iranian β-thalassemia major patients. Moreover, autoimmunization to RBC antigens was rare in our patients.
authors.affiliation: Department of Pediatrics, School of Medical Sciences, Zanjan University of Medical Sciences, Zanjan, Iran.
authors.forename: Kambiz
authors.initials: K
authors.lastname: Davari
authors.affiliation: Department of Laboratory Sciences, School of Paramedical Sciences, Zanjan University of Medical Sciences, Zanjan, Iran.
authors.forename: Mohammad Soleiman
authors.initials: MS
authors.lastname: Soltanpour
keywords: Alloantibody; autoantibody; b-thalassemia major; leukoreduced; leukoreduced packed red blood cells (RBCs)