Effects of prophylactic red blood cell (RBC) transfusion with extended antigen matching on alloimmunization in patients with Sickle Cell Disease (SCD).

BACKGROUND: RBC alloimmunization remains a significant problem for many patients with SCD. To reduce alloimmunization some strategies have been implemented to provide limited or extended antigen matched RBC transfusions to patients with SCD who need chronic transfusion support. The aim of this study was to evaluate the effects of prophylactic RBC transfusion with extended antigen matching on alloimmunization in patients with SCD. METHODS: This is a 20-year retrospective study of patients with SCD transfused with RBCS that were prospectively matched for D, C, c, E, e, K, Fya/Fyb, Jka/Jkb and S antigens. Our study included 95 patients, and none had antibodies documented before their first transfusion. Patients and donors were phenotyped and molecular typing was performed in all patients who had recent transfusions or a positive direct antiglobulin test to predict their antigen profile. Unexpected antibodies to the Rh system, meaning anti-Rh antibodies in patients whose serologic phenotype was Rh positive, were investigated by molecular genotyping for RH variant alleles. RESULTS: During this study-period, 12 (12.6%) were alloimmunized and 83 (87.4%) were not. Among the 12 patients who alloimmunized, 7 (58.3%) developed antibodies to Rh antigens and 5 (41.7%) produced antibodies to low prevalence antigens. All patients who developed Rh antibodies had RH variant alleles. Autoantibodies were found in 16 (16.8%) transfused patients. CONCLUSION: SCD patients benefit from receiving prophylactic RBC transfusions with extended antigen matching, as demonstrated by the reduction on the rates of alloimmunization and the lack of antibodies to K, FY, JK and S antigens, however, this strategy does not avoid alloimmunization to Rh and low-prevalence antigens.

Rh antibodies as a result of altered Rh epitopes on transfused red cells: a case series of seven Brazilian patients.

BACKGROUND: Rh antibodies produced by patients receiving Rh-matched RBC units may be associated with inheritance of altered RH alleles or a result of altered Rh epitopes on donor red blood cells (RBC). On this background, our aim was to evaluate unexpected Rh antibodies in Brazilian patients receiving regular transfusions and determine the clinical significance of the alloantibody produced. MATERIAL AND METHODS: We investigated seven patients (5 with sickle cell disease, 1 with myelodysplastic syndrome and 1 with ß-thalassaemia) with unexplained Rh antibodies. All patients had complete serological and molecular analyses. A lookback at the donor units transfused to these patients was performed and donors suspected of having Rh variants were recruited for further analysis. Laboratory and clinical findings were used to evaluate the clinical significance of the alloantibodies produced. RESULTS: The unexpected Rh antibodies found in the patients were not linked to the expression of partial Rh phenotypes according to serological and molecular analyses. Anti-D was found in two patients, anti-C was found in one patient, anti-c was found in one patient and anti-e was found in three patients carrying conventional D, C, c and e antigens respectively. Serological and molecular analyses of donors' samples revealed that six donors whose RBC were transfused to these patients carried partial Rh antigens. Only one anti-e in a patient with ß-thalassaemia was autoreactive and could not be explained by RH diversity in his donors. Three of the seven Rh antibodies were associated with laboratory and clinical evidence of a delayed haemolytic transfusion reaction or decreased survival of transfused RBC at first detection. DISCUSSION: Our study provides evidence that patients exposed to RBC units from donors with Rh variants may develop antibodies and some of these may be of clinical significance.