The Pharmacologic Rationale Supporting the Potential Use of Vepoloxamer for the Treatment of Acute Chest Syndrome in Patients with Sickle Cell Disease

Martin Emanuele
2016 Hematology Blood Transfusion & Disorders  
Sickle Cell Disease (SCD) is an autosomal recessive disorder caused by a single amino acid substitution in the Hemoglobin (Hb) β chain which causes it to polymerize in the deoxy state. Acute Chest Syndrome (ACS) with hypoxemic respiratory failure is a common cause of death in adult SCD subjects. Recent research has revealed how multiple disease processes including inappropriate cellular adhesion, hemolysis, dysfunctional coagulation, inflammation, and ischemia and tissue injury are involved in
more » ... CS pathophysiology. In this review article, we discuss how the activity of the highly purified non-ionic amphiphilic triblock copolymer vepoloxamer in the repair of damaged membranes and hindrance of inappropriate adhesive interactions and activation events potentially has utility in ACS, by virtue of its capacity for affecting multiple aspects of the pathophysiology.
doi:10.24966/hbtd-2999/100006 fatcat:srjqnulzbfbbdli2xh37k7j3g4