Lifelong aspirin even in childhood in sickle cell diseases

Abstract

Background: Sickle cell diseases (SCDs) are severe inflammatory processes on vascular endothelium, particularly at the capillary level since the capillary system is the main distributor of hardened red blood cells (RBCs) into the tissues.

Methods: All patients with the SCDs were included.

Results: We studied 222 males and 212 females with similar ages (30.8 vs 30.3 years, p>0.05, respectively). Disseminated teeth losses (5.4% vs 1.4%, p<0.001), ileus (7.2% vs 1.4%, p<0.001), cirrhosis (8.1% vs 1.8%, p<0.001), leg ulcers (19.8% vs 7.0%, p<0.001), digital clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (18.0% vs 13.2%, p<0.05), chronic renal disease (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were all higher but not acute chest syndrome (2.7% vs 3.7%), pulmonary hypertension (12.6% vs 11.7), deep venous thrombosis and/or varices and/or telangiectasias (9.0% vs 6.6%), and mean age of mortality (30.2 vs 33.3 years) in males (p>0.05 for all).

Conclusion: The hardened RBCs-induced capillary endothelial damage, inflammation, edema, and fibrosis are initiated at birth, and terminate with diffuse tissue hypoxia and multiorgan insufficiencies even at childhood in the SCDs. Although RBCs supports and corticosteroids in emergencies and hydroxyurea therapy in whole lifespan decrease severity of the SCDs with some extent, the survival still shortened in both genders, dramatically. Due to the higher mortality rates, lifelong aspirin with an anti-inflammatory dose should also be used even in childhood both to decrease severity of capillary endothelial inflammation and to prevent acute thromboembolic complications in SCDs.