P-083: IMPACT OF ALPHA THALASSEMIA COEXISTENCE ON TRANSFUSION NEED AMONG PATIENTS WITH SICKLE CELL DISEASE

Abstract

Purpose: Thalassemia alfa (Alpha-Thal) and sickle cell disease (SCD) are hemoglobinopathies that can be inherited concomitantly. The scientific literature suggests an improvement in the clinical picture of SCD when these conditions coexist, due to the greater potential for deformation of red blood cells and lower rates of hemolysis, positively impacting complications and transfusion needs. The aim of this study was to evaluate, in a population of patients with SCD, the frequency of deletions for the alpha-Thal genes and whether this characteristic resulted in a significantly lower number of transfusions. Materials and methods: 138 medical records of children born between 1998 and 2007, with SCD registered at Fundação Hemominas Juiz de Fora - Brazil, from August 1998 to July 2018 were evaluated. The inclusion criteria for the analysis were patients with active follow-up, diagnostic test through neonatal screening standardized (High Performance Liquid Chromatography- HPLC and Isoelectric Focusing Electrophoresis - IFE) by Núcleo de Ação e Pesquisa em Apoio Diagnóstico of Universidade Federal de Minas Gerais and the exclusion criteria were diagnosis through another methodology, loss of follow-up and death. The variables analyzed were the presence or absence of the deletion for Alpha-Thal(3,7) and the number of transfusions in the observational period using the Qui-Square test (SPSS 15 ®). Study approved by the Research Ethics Committee. Results: 10 patients died before the exams, there was a loss of follow-up in 11 patients due to the transfer or abandonment of treatment and 5 patients did not attend the testing for Alpha-Thal(3,7) in the period for collection. In the total sample, 60.9% were diagnosed with HbSS genotype, 28.3% HbSC, 6.5% HbSBeta0, 3.6% HbSBeta+ and 0.7% HbSD. 4.27% of the patients had two deletions, against 25.64% with one deletion and 65.81% without Alpha-Thal(3,7). Regarding polytransfused patients (10 or more transfusions), 22.1% of patients had no deletions, compared to 16.67% of those with a deletion and 0% of patients with two deletions for Alpha-Thal(3,7) (p = 0.036). Conclusion: It was observed a lower number of patients undergoing hypertransfusion and a lesser need for transfusions among those with deletions of Alpha-Thal(3,7) genes when comparing them to non-carriers, strengthening the role of Alpha Thalassemia coexistence as a protective factor in the SCD. The authors do not declare any conflict of interest