5610325 VARIABILITY OF OXYGENSCAN PARAMETERS ACCORDING TO THE TYPE OF THERAPY IN CHILDREN WITH SICKLE CELL DISEASE: PRELIMINARY DATA FROM A FEASIBILITY STUDY

Abstract

Background: Sickle Cell Disease (SCD) is characterized by significant phenotypic variability even when the same causative mutation is present (1). Validated biomarkers for routine use in SCD, to evaluate phenotypic severity and predict the evolution of organ damage and response to different therapies, are currently lacking. Oxygenscan (function of the instrument Laser-assisted Optical Rotational Red Cell Analyser) is a novel physiological assay of red blood cells’ deformability at different oxygen tensions; it allows the measurement of parameters such as maximum/minimum elongation index e point of sickling (2,3). Aims: This feasibility study aims at preliminarily evaluating the variability of the point of sickling, as a possible biomarker in SCD, with respect to different clinical conditions and types of treatment. Methods: We’ve prospectively gathered peripheral blood samples of pediatric patients with SCD followed in the Veneto Region Pediatric Sickle Cell Disease Reference Center of Padua University Hospital. The Oxygenscan test has been repeated twice for each sample. Results: We’ve analysed 67 blood samples of 48 pediatric patients with SCD (36 HbSS, 5 HbSbeta0, 7 HbSC). Patients were not receiving a disease-modifying therapy (n.11) or were on hydroxyurea (HU) (n.32), on HU and chronic transfusion regimen (n.2), HU and crizanlizumab (n.2), or after HSCT (n.2, one of them had been evaluated also before HSCT). Parameters obtained by Oxygenscan seem to vary according to the type of treatment received by patients (Figure 1).Conclusion: A higher number of samples and further analyses will be necessary to investigate a possible relationship between the point of sickling and clinical data such as the evolution of organ damage and response to different types of treatment.