Serum Visfatin Level in Sickle/β Thalassemia in Correlation with Frequency of Vaso Occlusion Crises

Abstract

Background: Hemoglobinopathies are the most common heritable disorders of erythrocytes, with sickle cell diseases (SCDs) and thalassemia being the most common. SCDs are characterized by the presence of sickle hemoglobin within red blood cell (RBC) as a result of point mutation (β6glu→val). SCDs include sickle cell anemia, hemoglobin C disease (HbSC), and sickle/β thalassemia. The latter is caused by coinheritance of two different mutations in β globin gene, one from each parent: one for sickle hemoglobin and the other for β thalassemia. Vaso occlusion is a key feature of this disease that occurs due to a series of interactions leading to painful crisis. Visfatin is a pro-inflammatory adipocytokine that contributes to vaso occlusive crises (VOC) through its role in the inflammatory process. Visfatin can directly promote endothelial dysfunction and stimulates vascular smooth muscle cells proliferation. Objectives: The primary aim of this study was to assess serum visfatin level in sickle/β thalassemia patients and evaluate the possible association between serum visfatin level in sickle/β thalassemia patients and the frequency of VOC, serum ferritin level, complete blood count (CBC) and high-performance liquid chromatography (HPLC) parameters. The secondary aim of this study was to compare the findings in two centers (Al-Karama Teaching Hospital, in Baghdad and Babylon Teaching Hospital for Maternity and Children, in Babylon). Materials and Methods: This was a cross-sectional study conducted from December 2018 until the end of August 2019 and included 77 individuals. Among them, 57 were sickle/β thalassemia patients (Group I): 28 from Al-Karama Teaching Hospital (Group IA) and 29 from Babylon Teaching Hospital for Maternity and Children (Group IB). The remaining 20 individuals act as a healthy control group (Group II). Clinical data were gathered, with collection of 5 mL of peripheral blood in order to examine CBC, C-reactive protein (CRP), serum ferritin, and serum visfatin. Results: Mean age was (13.14 ± 5.40) years. Males formed (59.74%), whereas females formed the remaining (40.26%). The mean number of annual frequency of VOC events was (3.05 ± 0.95) with no significant difference between the two subgroups. White blood cells count was significantly higher among cases compared to controls (P = 0.004). Hemoglobin was significantly lower among cases compared to controls (P < 0.001). Also, hemoglobin was significantly higher in cases of group IA compared to group IB with P = 0.022. Similarly, hemoglobin A2 (HbA2) was significantly higher in cases of group IA compared to group IB with P = 0.013. Serum ferritin was markedly higher among cases compared to controls with P < 0.001. Serum visfatin was significantly higher among cases compared to controls with P < 0.001. No significant difference was observed between the two subgroups regarding serum ferritin and visfatin.There was positive correlation between visfatin and annual frequency of VOC (r = 0.821, P < 0.001), moderate negative correlation between visfatin and HbA (r = –0.46, P < 0.001), moderate positive correlation between visfatin and HbS (r = 0.54 and P < 0.001), and strong positive correlation between visfatin and ferritin among cases group (r = 0.60 and P < 0.001) but not in control group. Conclusions: Serum visfatin level is significantly higher among patients with sickle/β thalassemia compared to healthy individuals with positive correlation exists between visfatin level and the annual frequency of VOC, ferritin level, and HbS; negative correlation with HbA, among those patients with sickle/β thalassemia. Hemoglobin level and HbA2 percentage significantly higher among Al-Karama hospital patients compared with those in Babylon hospital.