path(MESH:"Anemia, Sickle Cell")
The consequences of heme toxicity can be appreciated in hemolytic diseases such as β-thalassemia, sickle-cell disease (SCD), ischemia-reperfusion (IR), and malaria (Katori et al., 2002; Pamplona et al., 2007;Vinchi et al., 2013). PubMed:24904418
The most common pathological states in which RBCs interact with the endothelium include sickle cell disease [39], malaria [40], and diabetes [41]. PubMed:28458720
Studies of sickle cell anemia patients have reported decreased levels of Hpx following hemolysis [68]. PubMed:26368565
SCD and β-thalassemia are genetic diseases associated to erythrocytes that are prone to lysis due to defective Hb production (Heinle and Read, 1948; Pauling et al., 1949; Ingram, 1957; discussed later). PubMed:24904418
Hemolysis can happen due to ischemia/reperfusion, SCD or β-thalassemia. PubMed:24904418
SCD is a haemolytic disorder caused by a HBB (b-globin gene) mutation leading to polymerization of haemoglobin S, sickling, and haemolysis. PubMed:25307023
Multiple haemolytic disorders and therapeutic interventions produce substantial intravascular haemolysis. Examples include PNH, SCD, thalassaemias, glucose-6-phosphate dehydrogenase (G6PD) deficiency, hereditary spherocytosis and stomatocytosis, pyruvate kinase deficiency, autoimmune haemolytic anaemia, microangiopathies, acute haemolytic transfusion reactions, mechanical circulatory support [e.g., left ventricular assist device (LVAD)/extracorporeal membrane oxygenation (ECMO)], RBC transfusions and infusions of RBC substitutes. These disorders, therapies and procedures are also associated with an increased risk of thrombosis. PubMed:25307023
The consequences of heme toxicity can be appreciated in hemolytic diseases such as β-thalassemia, sickle-cell disease (SCD), ischemia-reperfusion (IR), and malaria (Katori et al., 2002; Pamplona et al., 2007;Vinchi et al., 2013). PubMed:24904418
SCD and β-thalassemia are genetic diseases associated to erythrocytes that are prone to lysis due to defective Hb production (Heinle and Read, 1948; Pauling et al., 1949; Ingram, 1957; discussed later). PubMed:24904418
Hemolysis can happen due to ischemia/reperfusion, SCD or β-thalassemia. PubMed:24904418
Multiple haemolytic disorders and therapeutic interventions produce substantial intravascular haemolysis. Examples include PNH, SCD, thalassaemias, glucose-6-phosphate dehydrogenase (G6PD) deficiency, hereditary spherocytosis and stomatocytosis, pyruvate kinase deficiency, autoimmune haemolytic anaemia, microangiopathies, acute haemolytic transfusion reactions, mechanical circulatory support [e.g., left ventricular assist device (LVAD)/extracorporeal membrane oxygenation (ECMO)], RBC transfusions and infusions of RBC substitutes. These disorders, therapies and procedures are also associated with an increased risk of thrombosis. PubMed:25307023
SCD is a haemolytic disorder caused by a HBB (b-globin gene) mutation leading to polymerization of haemoglobin S, sickling, and haemolysis. PubMed:25307023
Systemic hemolysis occurs during certain genetic and acquired anemia, such as in sickle cell disease and malaria. PubMed:26475040
Thus, in severe haemolytic diseases, such as paroxysmal nocturnal haemoglobinuria (PNH) and sickle cell disease (SCD), serum haptoglobin is typically undetectable and plasma haemoglobin is elevated (Tabbara, 1992). PubMed:25307023
Thus, in severe haemolytic diseases, such as paroxysmal nocturnal haemoglobinuria (PNH) and sickle cell disease (SCD), serum haptoglobin is typically undetectable and plasma haemoglobin is elevated (Tabbara, 1992). PubMed:25307023
Multiple haemolytic disorders and therapeutic interventions produce substantial intravascular haemolysis. Examples include PNH, SCD, thalassaemias, glucose-6-phosphate dehydrogenase (G6PD) deficiency, hereditary spherocytosis and stomatocytosis, pyruvate kinase deficiency, autoimmune haemolytic anaemia, microangiopathies, acute haemolytic transfusion reactions, mechanical circulatory support [e.g., left ventricular assist device (LVAD)/extracorporeal membrane oxygenation (ECMO)], RBC transfusions and infusions of RBC substitutes. These disorders, therapies and procedures are also associated with an increased risk of thrombosis. PubMed:25307023
Studies of sickle cell anemia patients have reported decreased levels of Hpx following hemolysis [68]. PubMed:26368565
The most common pathological states in which RBCs interact with the endothelium include sickle cell disease [39], malaria [40], and diabetes [41]. PubMed:28458720
The preferential adaptive increase in cerebral blood flow has also been demonstrated in other models of anemia, including acute hemodilution and chronic sickle cell anemia (3, 41). PubMed:29351418
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If you find BEL Commons useful in your work, please consider citing: Hoyt, C. T., Domingo-Fernández, D., & Hofmann-Apitius, M. (2018). BEL Commons: an environment for exploration and analysis of networks encoded in Biological Expression Language. Database, 2018(3), 1–11.