Haematologica

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