Frequency of pain crises in sickle cell anemia and its relationship with the sympatho-vagal balance, blood viscosity and inflammation

Abstract

BackgroundRecent evidence suggests that autonomic nervous system activity could be involved in the pathophysiology of sickle cell disease, but it is unclear whether differences in autonomic nervous system activity are detectable during steady state in patients with mild and severe disease. The aim of the present study was to compare the autonomic nervous system activity, blood rheology, and inflammation in patients with sickle cell anemia according to the frequency of acute pain crisis. Design and MethodsTwenty-four healthy volunteers, 20 patients with sickle cell anemia with milder disease, and 15 patients with sickle cell anemia with more severe disease were recruited. Milder disease was defined as having no pain crisis within the previous year. More severe disease was defined as having had within the previous year three or more pain crises which were documented by a physician and required treatment with narcotics. The autonomic nervous system activity was determined by spectral analysis of nocturnal heart rate variability. Blood viscosity determination and measurements of several inflammatory markers (interleukin-6, soluble vascular cell adhesion molecule-1, soluble CD40 ligand and sL-selectin) were made on blood samples collected in steady-state conditions. ResultsResults showed that: 1) patients who had suffered more frequent pain crises had lower parasympathetic activity and greater sympatho-vagal imbalance than both controls and patients with milder disease. However, when adjusted for age, no significant difference was detected between the two sickle cell anemia patient groups; 2) patients who had suffered more frequent pain crises had higher blood viscosity than patients with milder disease, and this was not dependent on age. ConclusionsResults from the present study indicate that both the autonomic nervous system activity and blood viscosity are impaired in patients with sickle cell anemia exhibiting high frequency of pain crisis in comparison with those who did not experience a crisis within the previous year. Haematologica 2011;96(11):1589-1594. doi:10.3324/haematol.2011 This is an open-access paper. Frequency of pain crises in sickle cell anemia and its relationship with the sympatho-vagal balance, blood viscosity and inflammation

Hemorheological risk factors of acute chest syndrome and painful vaso-occlusive crisis in children with sickle cell disease

“…These findings agree well with those described in SCA adults by several authors who observed that higher VOC frequency was associated with higher Hct, 12 blood viscosity 11 and RBC deformability. 13,28 Ballas et al 13 proposed that the more sickle RBC are deformable, the greater their adherence to vascular endothelium will be, and the more they may cause VOC.…”

“…The lack of association between RBC deformability and VOC categories may be due to the very wide inter-individual variability of this parameter, as previously shown by Ballas et al 13 Taken altogether, our results indicate that blood viscosity may be a useful marker of increased risk of VOC in SCA children, as it is in SCA adults. 11 Castro et al 30 previously reported that reduced steadystate Hb and increased HbF levels decreased the ACS rate in SCA patients (adults and children together). In the present study, we did not find any significant difference in the Hb, Hct or HbF levels between ACS-SCA and NACS-SCA children.…”

“…8 In addition, large RBC aggregates increase the resistance to blood flow in smaller vessels if they are not disaggregated before entering into the capillary network. 10 Previous studies in adult SCA patients demonstrated that increased blood viscosity 11,12 and increased RBC deformability 13 are risk factors for VOC. Whether these hemorheological parameters are also involved in the pathophysiology of VOC in SCA children is unknown.…”

Coronary Artery Ectasia in Atherosclerotic Coronary Artery Disease, Inflammatory Disorders, and Sickle Cell Disease

“…It is essential to assess the percentage of Hb S to determine the severity of the disease and risk for complications. Additionally, it is important to inquire about the number and frequency of vaso-occlusive crises, because these may reflect increased blood viscosity and a more severe inflammatory process [72]. Such information can help the clinicians determine the type of antithrombotic therapies and their potential benefits if deemed needed.…”

Using the hemoglobin switch for the treatment of sickle cell disease

“…3 Sickle cell disease is thus caused by mutations in the hemoglobin beta gene that only become apparent after the switch from fetal hemoglobin (using the gamma gene) to adult hemoglobin (using the beta gene). Reactivation of the fetal form of the complex could potentially solve or decrease the 'sickle problem', since the fetal hemoglobin gamma could replace the mutated hemoglobin beta.…”