Innate immune cells, major protagonists of sickle cell disease pathophysiology

Haematologica. 2020 Jan 31;105(2):273-283. doi: 10.3324/haematol.2019.229989. Print 2020.

Abstract

Sickle cell disease (SCD), considered the most common monogenic disease worldwide, is a severe hemoglobin disorder. Although the genetic and molecular bases have long been characterized, the pathophysiology remains incompletely elucidated and therapeutic options are limited. It has been increasingly suggested that innate immune cells, including monocytes, neutrophils, invariant natural killer T cells, platelets and mast cells, have a role in promoting inflammation, adhesion and pain in SCD. Here we provide a thorough review of the involvement of these novel, major protagonists in SCD pathophysiology, highlighting recent evidence for innovative therapeutic perspectives.

Publication types

  • Review

MeSH terms

  • Anemia, Sickle Cell* / therapy
  • Humans
  • Immunity, Innate
  • Inflammation
  • Neutrophils
  • Pain