Heme-mediated cell activation: the inflammatory puzzle of sickle cell anemia

作者：da Guarda Caroline Conceicao; Santiago Rayra Pereira; Fiuza Luciana Magalhaes; Aleluia Milena Magalhaes; Dutra Ferreira Junia Raquel; Boas Figueiredo Camylla Vilas; Modeste Alexandre Yahouedehou Setondji Cocou; de Oliveira Rodrigo Mota; Lyra Isa Menezes; Goncalves Marilda de Souza

来源：Expert Review of Hematology, 2017, 10(6): 533-541.

DOI：10.1080/17474086.2017.1327809

• Inflammation
• heme
• hemolysis
• sickle cell anemia
• sickle cell disease

Introduction: Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules.Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA.Expert commentary: Free Hb, heme and iron, potent cytotoxic intravascular molecules released during hemolysis, can exacerbate, modulate and maintain the inflammatory response, a main feature of SCA. Endothelial cells in the vascular environment, as well as leukocytes, can become activated via the molecular signaling effects of heme. Due to the hemolytic nature of SCA, hemolysis represents an interesting therapeutic target for heme-scavenging purposes.

• 出版日期2017