Sickle Cell Anemia Thalassemia Research Articles

Investigation of Some Trace Elements levels & Antioxidants in Patients with Thalassemia Major and Sickle Cell AnemiaThalassemia in South of Iraq

Back ground: Thalassemia major) THM( is the most common single gene disorder. It is a hereditary blooddisease characterized by reduced or absent production of beta globin chains. For the patients with SCA-Thal,they have two mutations (sickle cell anemia and thalassemia). The ratio of minerals and trace elements in thepatients’ blood played an important role in many biological processes. This study aims to measure the levelof seven elements, Fe, Mn, Ca+2, Mg, P, Cr, Co and three another antioxidant element (Zn, Cu &Se) in theblood of THM and SAC-thal patients.Results: In THM and SCA-Thal patients, antioxidant elements (Cu, Zn & Se), showed highly significantbehaviour when compared to each other and with the control group as total patient population, and as (total,male and female) numbers. In thalassemia patients, Fe, P & Co also showed highly significant behaviour,while Ca + 2 behaviour was not significant for male numbers only. Elemental Mg & Cr, demonstratedinsignificant behaviours. In SCA-Thal patients, Mn & Ca + 2 showed highly significant behaviour, while,Fe showed a non-significant behaviour relative to male numbers only, and Mg, P, Cr & Co showed nonsignificant behaviour.Conclusion: The study revealed the similarity of antioxidant elements behaviour in both diseases, as well asthe statistical behaviour of all elements when compared with the control group for these diseases.

Immunisation anti-érythrocytaire dans les hémoglobinopathies : à propos de 84 cas

AimsTo estimate the rate of red cell immunization in hemoglobinopathies. Patients and methodsProspective 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. ResultsThe 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). ConclusionThis study proves the interest of using RH-KELL red cell units compatible in patients with hemoglobinopathies in order to reduce alloimmunisation rates.