One of transfusion's side effects is alloimmunization against red blood cell (RBC) antigens. Early diagnosis by antibody screening is an important step in the detection of these alloantibodies. The authors studied the frequency of alloimmunization in thalassemic patients of 4 centers (2 adult and 2 pediatric centers) and compared the rates in children (up to 15 years) and adults. Antibody screening tests were performed by gel method according to its standard pattern and respective program. In positive cases, antibody identification test by gel method was performed. Eight hundred thirty-five patients were studied; 548 (65.6%) were adults (mean age = 24.5), and 287 (34.4%) cases were pediatrics (mean age = 10.05). Of these patients, 74.1% had no history of transfusion reaction, whereas 21 (2.5%) had hemolytic complications. Seventy-eight (9.3%) exhibited allergic symptoms, and 117 (14%) cases experienced febrile reactions during transfusion. Antibody screening showed positive results in 22 pediatric cases (7.7%) and 79 adults (14.4%); 72 (71.3%), 19 (18.8%), 3 (3%), and 1 (1%) cases exhibited single, double, triple, and autoantibodies, respectively. Anti-Kell antibody was seen in 34 (33.7%) cases, anti-D was seen in 11 (10.9%) cases, and anti-E in was seen in 10 (9.9%) cases. The authors observed 8 anti-D+C (7.9%) cases, 1 anti-D+E (1%), 3 anti-Kell+E, 3 anti-Kell+Kpa (3%), and 1 anti-Kell+D double antibodies. These antibodies were also a combination of Rh subgroups or Rh and Kell subgroups. The authors observed meaningful relations between history of transfusion reactions and age with antibody screening results (P = .005). Based on alloantibodies types, more than two thirds of them were Rh subgroups and Kell groups. Phenotype determination of RBCs before beginning chronic blood transfusion and careful cross-matching with Kell and Rh subgroups in addition to ABO may help reduce alloimmunization in chronic transfusion patients.
findings provide further evidence for a high prevalence of VTE in critically ill patients with SARS-CoV-2 infection. These findings are important for identifying a high risk group for adverse outcomes and to raise clinicians' awareness of VTE risk amongst critically ill COVID-19 patients.
Hydroxyurea (HU), a drug which can reactivate fetal hemoglobin (Hb F) production, is frequently prescribed to β-thalassemia (β-thal) patients. However, transfusion requirements of only a subset of patients are reduced upon HU treatment. Because of its potential side-effects, targeted prescription of HU is imperative. To identify genetic markers that correlate with drug response, we have carried out a retrospective association study of single nucleotide polymorphisms (SNPs) in three Hb F quantitative trait loci (QTLs): the XmnI polymorphism, BCL11A, and the HBS1L-MYB intergenic region, with the response to HU in a cohort of 81 transfusion-dependent Iranian β-thal patients. An increase in blood transfusion intervals post-therapy was used to measure drug response. Our results suggest that presence of the XmnI T/T genotype or the BCL11A rs766432 C allele correlates strongly with response to HU (p <0.001). Accordingly, these markers may be used to accurately predict the HU response of Iranian β-thal patients.
There is a need for higly accurate non-invasive methods for assessing organ iron content in thalassaemia patients. This study evaluated the relation between serum ferritin level, liver enzyme levels and hepatitis C antibody and liver and heart iron deposition assessed by MRI T2*. Data were obtained from the medical records of 156 thalassemia major patients in Tehran. There was a moderate negative correlation between serum ferritin and liver MRI T2* relaxation time (r = -0.535) and a weak negative correlation between serum ferritin and heart MRI T2* relaxation time (r = -0.361). Hepatitis C infection and liver enzyme levels did not confound or modify the relation between ferritin and liver or heart MRI T2*. Liver and heart MRI T2* readings were poorly correlated (r = 0. 281). Routine evaluation of liver and heart iron content using MRI T2* is suggested to better evaluate the haemosiderosis status in thalassemia patients. Relation entre la ferritine sérique et l'IRM hépatique et cardiaque pondérée en T2* chez des patients atteints de bêta-thalassémie majeure RÉSUMÉ Des méthodes non-invasives de haute précision sont nécessaires pour l'évaluation de la concentration en fer dans les organes des patients atteints de thalassémie. La présente étude a évalué la relation entre le taux de ferritine sérique, les taux d'enzymes hépatiques, la présence d'anticorps anti-hépatite C et les dépôts de fer dans le foie et le coeur examinés par IRM pondérée en T2*. Les données ont été obtenues à partir des dossiers médicaux de 156 patients atteints de thalassémie majeure à Téhéran. On a observé une corrélation négative modérée entre la ferritine sérique et le temps de relaxation T2* de l'IRM hépatique (r = -0,535) ainsi qu'une faible corrélation négative entre la ferritine sérique et le temps de relaxation T2* de l'IRM cardiaque (r = -0,361). L'infection par le virus de l'hépatite C et les taux d'enzymes hépatiques ne constituaient pas de facteurs de confusion ou de modification de la relation entre la ferritine et l'IRM hépatique ou cardiaque pondérée en T2*. La corrélation entre les résultats de l'IRM hépatique et de l'IRM cardiaque pondérées en T2* était médiocre (r = 0,281). L'évaluation systématique de la concentration de fer dans le foie et le coeur à l'aide de l'IRM pondérée en T2* semble produire une meilleure évaluation de la présence d'une hémosidérose chez les patients atteints de thalassémie.
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