Autosplenectomy may be a good prognostic sign in sickle cell diseases

Abstract

Background: Sickle cell diseases (SCDs) are inborn and destructive processes on vascular endothelium, particularly at the capillaries. Methods: Patients with red blood cells (RBCs) transfusions of less than 50 units in their lives were put into the first and 50 units or higher were put into the second groups. Results: There were 224 patients in the first and 92 patients in the second groups. Mean ages were similar in them (28.9 vs 30.0 years, respectively, p>0.05). The male ratio was higher in the second group (45.5% vs 64.1%, p<0.001). In contrast to the lower prevalence of autosplenectomy (56.2% vs 45.6%, p<0.05), painful crises per year, digital clubbing, chronic obstructive pulmonary disease (COPD), leg ulcers, stroke, chronic renal disease (CRD), and coronary heart disease (CHD) were all higher in the second group (p<0.05 for all). 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. In contrast to the lower prevalence of autosplenectomy, painful crises per year, digital clubbing, COPD, leg ulcers, stroke, CRD, and CHD were higher in the second group. So there may be an inverse relationship between prevalence of autosplenectomy and severity of SCDs, and spleen may act as a chronic inflammatory focus as a filter of blood for these abnormally hardened RBCs. Key words: Sickle cell diseases, sickled or just hardened red blood cells, capillary endothelial edema, myocardial infarction, stroke, sudden deaths, autosplenectomy