SCD silent damage is progressive, independent of the
occurrence of vaso-occlusive crises (VOCs).
Hemoglobin S (HbS) polymerization drives hemolysis and anemia, which can lead to vasculopathy, impaired oxygen delivery, and further downstream complications, including long-term organ damage.
HbS polymerization is the pathologic process at the source of red
blood cell (RBC) sickling. Left unchecked, this sickling can lead to
subsequent hemolysis, anemia, and vaso-occlusion, and can lead to
further downstream consequences.5-8
See its impact on SCD progression.
Listen to an expert in the field of SCD talk about how HbS polymerization drives hemolysis and anemia, which can lead to further complications.1-5
References: 1. Telen MJ, Malik P, Vercellotti GM. Therapeutic strategies for sickle cell disease: towards a multi‐agent approach. Nat Rev Drug Discov. 2019;18(2):139-158. doi:10.1038/s41573-018-0003-2 2. Kato GJ, Piel FB, Reid CD, et al. Sickle cell disease. Nat Rev Dis Primers. 2018;4:18010. doi:10.1038/nrdp.2018.10 3. Kato GJ, McGowan V, Machado RF, et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006;107(6):2279-2285. doi:10.1182/blood-2005-06-2373 4. Damanhouri GA, Jarullah J, Marouf S, Hindawi SI, Mushtaq G, Kamal MA. Clinical biomarkers in sickle cell disease. Saudi J Biol Sci. 2015;22(1):24-31. doi:10.1016/j.sjbs.2014.09.005 5. Kato GJ, Steinberg MH, Gladwin MT. Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest. 2017;127(3):750-760. doi:10.1172/JCI89741 6. Ataga KI, Gordeuk VR, Allen IE, Colby JA, Gittings K, Agodoa I. Low hemoglobin increases risk for stroke, kidney disease, elevated pulmonary artery systolic pressure, and premature death in sickle cell disease: a systematic literature review and meta-analysis. PLoS One. 2020;15(4):e0229959. doi:10.1371/journal.pone.0229959 7. Gordeuk VR, Castro OL, Machado RF. Pathophysiology and treatment of pulmonary hypertension in sickle cell disease. Blood. 2016;127(7):820‐828. doi:10.1182/blood-2015-08-618561 8. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364(9442):1343-1360. doi:10.1016/S0140-6736(04)17192-4