SCD silent damage is progressive, independent of the occurrence of vaso-occlusive crises (VOCs). Hemoglobin S (HbS) polymerization drives hemolysis and anemia, that can lead to vasculopathy and organ damage.1-5
A recent meta-analysis explored the impact of hemoglobin (Hb) levels in patients with SCD.6
HbS polymerization is the underlying pathologic process that initiates red blood cell (RBC) sickling and subsequent hemolysis, anemia, and vaso-occlusion.5-7 These pathologic processes underlie the long-term tissue damage of SCD.5,7,8
See its impact on SCD progression.
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. 2. Kato GJ, Piel FB, Reid CD. Sickle cell disease. Nat Rev Dis Primers. 2018;4(article 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. 4. Damanhouri GA, Jarullah J, Marouf S, Hindawi SI, Mustaq G, Kamal MA. Clinical biomarkers in sickle cell disease. Saudi J Biol Sci. 2014;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. 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. Blood. 2018;132(suppl 12):1-12. 7. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet. 2004;364(9442):1343-1360. 8. Gordeuk VR, Castro OL, Machado RF. Pathophysiology and treatment of pulmonary hypertension in sickle cell disease. Blood. 2016;127(7):820‐828.