Acute painful crises may be causes of sudden deaths in sickle cell diseases

• DOI: 10.5742/mewfm.2023.95256109
• Publication Date: 2023-06-01T05:59:22Z
• Authors: Mehmet Rami Helvaci; Mehpare Camlibel; Burcu Yuksek; Alper Sevinc; Celaletdin Camci; Abdulrazak Abyad; Lesley Pocock

Background: Sickle cell diseases (SCDs) are severe inflammatory processes on vascular endothelium, especially at the capillary level because the capillary system is the actual distributor of hardened red blood cells (RBCs) into the tissues. Methods: All cases with the SCDs were included. Results: We studied 222 males and 212 females with similar mean 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 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 hardened RBCs-induced capillary endothelial damage, inflammation, edema, and fibrosis are initiated at birth, and terminate with disseminated tissue hypoxia and multiorgan failures even at childhood in the SCDs. Although RBCs supports and corticosteroids in acute phase and aspirin plus hydroxyurea both in acute and chronic phases decrease severity of the diseases, 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 acute painful crises, multiorgan failures, and sudden deaths. Key words: Sickle cell diseases, acute painful crises, sudden deaths, capillary endothelial inflammation, capillary endothelial edema, atherosclerosis, aging