Effects of Age and Sex on Sickle Cell Disease Avascular Necrosis

Abstract

Introduction: Sickle cell disease (SCD) is a hemoglobinopathy. Based on genotypes, it is classified into sickle cell thalassemia (SCTh) and sickle cell anemia (SCA). Red blood cells in SCD are less pliable; therefore, these cells distort to a sickle shape that leads to tissue infarctions and progressive bone avascular necrosis (AVN). Aim: To study the significant effects of age and sex on AVN in SCD. Methods: Data of 68 patients with SCD were collected (25 and 43 patients with SCTh and SCA, respectively). Their age ranged from 4 to 78 years. Statistical test conducted to analyze the significant effects of age and sex on AVN in all the 68 patients. Results: AVN prevalent is 76% and 72% in patients with SCTh and SCA, respectively. In SCTh age effect on AVN in SCTh (p=0.546) and Sex effect on AVN (p=0.013), while in SCA age effect on AVN (p=0.783) and Sex effect on AVN in SCA (p=0.334). The HPLC results showed HbF mean values in SCTh 12%, while in SCA 17%. HPLC results showed HbA2 mean values in SCTh 4.9% (4.9 in children, 4.9 in adults, 4.8 in females and 4.9 in males), and in SCA 2.1% (1.9 in children, 2.4 in adults, 2.1 in females and 2.3 in males. Conclusion: In age group, no significant relation found between age and AVN in both SCTh and SCA. In sex group no significant relationship found between sex and SCA. However, a significant result detected between AVN and sex in SCTh; males have more tendency to develop AVN than females in both SCTh and SCA. HbF was higher in SCA than in SCTh due to hydroxyurea treatment and AVN prevalence was also less in SCA.