Innate immune cells, major protagonists of sickle cell disease pathophysiology
Slimane Allali,
Thiago Trovati Maciel,
Olivier Hermine,
Mariane de Montalembert
Affiliations
Slimane Allali
Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker Hospital for Sick Children, Assistance Publique – Hôpitaux de Paris (AP-HP), Paris Descartes University, Paris;Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, Paris Descartes – Sorbonne Paris Cite University, Imagine Institute, Inserm U1163, Paris;Laboratory of Excellence GR-Ex, Paris
Thiago Trovati Maciel
Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, Paris Descartes – Sorbonne Paris Cite University, Imagine Institute, Inserm U1163, Paris;Laboratory of Excellence GR-Ex, Paris
Olivier Hermine
Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, Paris Descartes – Sorbonne Paris Cite University, Imagine Institute, Inserm U1163, Paris;Laboratory of Excellence GR-Ex, Paris;Department of Hematology, Necker Hospital for Sick Children, AP-HP, Paris Descartes University, Paris, France
Mariane de Montalembert
Department of General Pediatrics and Pediatric Infectious Diseases, Reference Center for Sickle Cell Disease, Necker Hospital for Sick Children, Assistance Publique – Hôpitaux de Paris (AP-HP), Paris Descartes University, Paris;Laboratory of Excellence GR-Ex, Paris
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.
