Advances in clinical research in sickle cell disease

Abstract

Sickle cell disease continues to cause significant morbidity and mortality despite increases in life expectancy. This review looks at recent translational research in sickle cell disease, covering the red cell membrane, the vascular endothelium, local and systemic inflammation and the potentially pivotal role of nitric oxide as a key regulator of sickle cell complications. Clinical research reviewed includes pulmonary hypertension, which is emerging as a particularly deadly scourge of adults with any haemolytic anaemia, and newer insights into older, more established complications and treatments, such as red cell transfusions, the need for and use of iron chelation and avascular necrosis. Finally, recent studies about those factors that affect the day-to-day lives of persons with sickle cell disease, pain and neuropsychiatric functioning, are discussed.