Bart J. Biemond

Abstract Sickle cell disease (SCD) is caused by hemoglobin S, which alters red blood cells (RBCs) rheological properties leading to vaso-occlusion and chronic hemolysis, the main hallmarks of the disease. They drive the broad clinical spectrum of SCD, including acute complications and progressive organ damage. Oxygen gradient ektacytometry (oxygenscan) is a functional assay that measures RBC deformability and sickling tendency during an oxygen gradient.

1 Introduction Sickle cell disease (SCD) is a chronic, multi-systemic disease that significantly impacts both patients' and global health. SCD is caused by autosomal recessive mutations in the HBB gene and is characterized by chronic hemolytic anemia, recurrent painful vaso-occlusive ischemic events (VOE), and systemic inflammation [1-3]. These processes contribute to progressive organ damage and reduce life expectancy in patients with SCD.

ANGPT-1 angiopoietin-1 ANGPT-2 angiopoietin-2 CD200R1 OX-2 membrane glycoprotein (CD200, MOX1) cell surface glycoprotein CD200 receptor 1 CXCL1 growth-regulated alpha protein CXCL5 C-X-C motif chemokine 5 CXCL6 C-X-C motif chemokine 6 EGLN1 Egl nine homolog 1 EPO erythropoietin G-CSF granulocyte colony-stimulating factor G-CSFR granulocyte colony-stimulating factor receptor IL-10 interleukin-10 IL-18 interleukin-18 IL-1RN interleukin-1 receptor antagonist protein IL-1β interleukin-1 beta IL-6...