[Red blood cell immunization in haemoglobinopathie: about 84 cases]. / Immunisation anti-érythrocytaire dans les hémoglobinopathies : à propos de 84 cas.

AIMS: To estimate the rate of red cell immunization in hemoglobinopathies. PATIENTS AND METHODS: Prospective study (1990-2009) about 84 patients: 44 homozygous sickle cell anemia, one heterozygous sickle cell anemia S/C, 30 thalassemia and nine sickle cell anemia-thalassemia. The mean age was 10.13 years (extremes: 1-45). The red cell units transfused were ABORH1 compatible, then RH-KELL phenotyped after 2006 and phenocompatible after alloimmunisation. The cross-match was realized using indirect antiglobuline test. Irregular red cell antibody screening was realized before every transfusional episode and the direct antiglobuline test was done when there was a poor transfusional efficiency. RESULTS: The number of red blood cells units transfused was 3545 (42.2/patient). The number of red cell antibody screening and the number of direct antiglobulin test were respectively 1474 (17.5/patient) and 272 (3.2/patient). Twenty-seven antibodies were identified (32.1%): 14 alloantibodies (16.6%, 16.6% in sickle cell disease, 16.6% in thalassemia, P=1), 16 antoantibodies (19.04%, 11.1% in sickle cell disease, 33.3% in thalassemia, P=0.018). There were three cases of association of allo- and autoantibodies. The most frequent alloantibodies were anti-RH3 and anti-KEL1 and were developed after transfusion of standard red cell units. There was no significant relation, neither between sex and risk of immunization, nor between the number of red cell units transfused and alloimmunization. On the other hand, there was a significant relation between autoimmunization and the number of red cell units transfused in thalassemia (P<0.001). CONCLUSION: This study proves the interest of using RH-KELL red cell units compatible in patients with hemoglobinopathies in order to reduce alloimmunisation rates.