Abstract

Background: Sickle cell diseases (SCDs) are inborn and destructive processes on vascular endothelium, particularly at the capillaries. 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). Although the higher prevalence of autosplenectomy (50.4% vs 53.3%, p<0.05), transfused units of red blood cells (RBCs) in their lives (48.1 vs 28.5, p=0.000), 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), clubbing (14.8% vs 6.6%, p<0.001), coronary heart disease (CHD) (18.0% vs 13.2%, p<0.05), chronic renal disease (CRD) (9.9% vs 6.1%, p<0.05), chronic obstructive pulmonary disease (COPD) (25.2% vs 7.0%, p<0.001), and stroke (12.1% vs 7.5%, p<0.05) were all lower in females. Conclusion: The sickled or just hardened RBCs-induced capillary endothelial damage initiates at birth, and terminates with multiorgan failures even at childhood. Although RBCs suspensions and corticosteroids in acute, and aspirin with an anti-inflammatory dose plus low-dose warfarin plus hydroxyurea both in acute and chronic phases decrease severity, survivals are still shortened in both genders, dramatically. Although the higher prevalence of autosplenectomy, transfused units of RBCs in their lives disseminated teeth losses, ileus, cirrhosis, leg ulcers, clubbing, CHD, CRD, COPD, and stroke were all lower in females. So there may be an inverse relationship between autosplenectomy and severity of SCDs, and spleen may act as a chronic inflammatory focus as a filter of blood for these sickled or just hardened RBCs. Key words: Sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, autosplenectomy, female gender

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