An exaggerated capillary endothelial edema may be the cause of sudden deaths in sickle cell diseases

Abstract

Background: Sickle cell diseases (SCDs) are inborn and severe inflammatory processes on vascular endothelium, particularly at the capillaries which are the actual distributors of the sickled or just hardened red blood cells (RBCs) into the tissues. Methods: All patients of 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 higher in males 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%), or mean age of mortality (30.2 vs 33.3 years) (p>0.05 for all). Conclusion: The sickled or just hardened RBCs-induced capillary endothelial damage, inflammation, edema, and fibrosis are initiated at birth, and terminate with disseminated tissue hypoxia, multiorgan failures, and sudden deaths even at childhood. Although RBCs suspensions and corticosteroids in acute and aspirin plus hydroxyurea in acute and chronic phases decrease severity of the destructive process, survivals are still shortened in both genders, dramatically. Infections, medical or surgical emergencies, or emotional stresses-induced increased basal metabolic rate aggravates the sickling and capillary endothelial edema, and may terminate with multiorgan failures-induced sudden deaths in the SCDs. Key words: Sickle cell diseases, sickled or just hardened red blood cells, capillary endothelial damage, exaggerated capillary endothelial edema, sudden deaths

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