Abstract
Introduction: Risk factors for neurological complications in sickle cell disease differ in the adult and pediatric populations. Here, we focused on neurological complications in adults with sickle cell disease.Methods: Patients were selected using the audit data from the St George's Hospital Red Cell Database. The genotyping, demographics, clinical data, and investigation findings were collected.Results: A total of 303 patients were enrolled in the study: hemoglobin S homozygosity (HbSS) genotype 56%, hemoglobin S and C coinheritance (HbSC) genotype 35%, and hemoglobin S and β-thalassemia coinheritance (HbSβ) thalassemia genotype 9%; the mean age was 38.8 years (±13.5 SD) with 46% males. The most common neurological complication was cerebrovascular disease (n = 37, 12%) including those with ischemic stroke (10%), cerebral vasculopathy (3%), and intracranial hemorrhage (1%). Ischemic stroke was common among the HbSS genotype compared with other genotypes (8 vs. 1.6%, p = 0.001). Comparing the patients with sickle cell disease who had suffered a stroke to those who had not, there was a higher proportion of intracranial vasculopathy (p = 0.001, in particular, Moyamoya) and cognitive dysfunction (p < 0.0001).Conclusion: Our cohort supports previous reports that the most common neurological complication in adult sickle cell patients is cerebrovascular disease. Strategies to prevent cerebral vasculopathy and cognitive impairment should be explored.
Highlights
Risk factors for neurological complications in sickle cell disease differ in the adult and pediatric populations
There is no consensus about the classification of HbS/ß-thalassemia, but Neurological Complications in sickle cell disease (SCD)
We included: [1] patients with SCD defined as Hemoglobin S homozygosity (HbSS), Hemoglobin S and C coinheritance (HbSC), HbSβ0, and HbSβplus thalassemia genotypes confirmed with hemoglobin analysis and other confirmatory documentation of phenotype; [2] patients ≥ 18 years; [3] patients with any infarct status including none, childhood or adult-onset, silent cerebral infarcts or overt stroke; [4] patients with any therapy modality for stroke prevention
Summary
Risk factors for neurological complications in sickle cell disease differ in the adult and pediatric populations. We focused on neurological complications in adults with sickle cell disease. Sickle cell disease is an autosomal recessive disease due to hemoglobin S homozygosity (HbSS) or coinheritance with other abnormal hemoglobins including hemoglobin C (HbSC) or β-thalassemia (HbS/ß-thalassemia). There is no consensus about the classification of HbS/ß-thalassemia, but Neurological Complications in SCD it is usually classified in two types: HbSβ0 and HbSβplus thalassemia [4]. Both HbSS and HbSβ0 genotypes have severe sickle cell disease (SCD) expression and are usually studied in combination [5]. Hemoglobin S homozygosity is less soluble than normal hemoglobin, leading to microcirculation impairment, end-organ ischemia, and necrosis
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