Profile of crizanlizumab and its potential in the prevention of pain crises in sickle cell disease: evidence to date.

ABSTRACT: Sickle cell disease (SCD) is one of the most common inherited blood disorders globally. It is a grouping of autosomal recessive genetic disorders identified by a genetic mutation that replaces glutamic acid with valine at the sixth amino acid on the hemoglobin β-globin chain. Millions of people around the world live with a severe genotype of SCD that is often associated with occlusion of the microvasculature resulting in episodes of severe pain and multiple organ system dysfunction. These episodes, commonly categorized as vaso-occlusive crises (VOC), are a distinctive clinical presentation of SCD which represents the majority of SCD morbidity and associated hospitalizations. Though the complete process by which these crises occur is complex and not fully outlined, evidence reveals this pro

SUBMITTER: Riley TR 

PROVIDER: S-EPMC6720158 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature