Pathophysiological insights in sickle cell disease

Indian Journal of Medical Research

Volumn 134, Issue 10, 2011, Pages 532-537

  Odièvre, Marie Hélène ^a   Verger, Emmanuelle ^a   Silva Pinto, Ana Cristina ^b   Elion, Jacques ^a  

^a INSERM   (France)

^b UNIVERSITÉ DES ANTILLES ET DE LA GUYANE   (France)

Author keywords

Cell activation; Cell adhesion; Haemoglobin S; Inflammation; Ion channels; Nitric oxide; Sickle cell disease

Indexed keywords

ENDOTHELIN 1; HEMOGLOBIN S; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN; NITRIC OXIDE; PHOSPHATIDYLSERINE; THROMBOSPONDIN; VASCULAR CELL ADHESION MOLECULE 1;

ACUTE CHEST SYNDROME; BLOOD VESSEL TONE; CELL ADHESION; CONFORMATIONAL TRANSITION; DEHYDRATION; ENDOTHELIUM; ERYTHROCYTE; ERYTHROPHAGOCYTOSIS; FEMUR HEAD NECROSIS; HEMOLYSIS; HUMAN; HYDROPHOBICITY; MICROCIRCULATION; NEUTROPHIL; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PROTEIN POLYMERIZATION; PULMONARY HYPERTENSION; REVIEW; SICKLE CELL ANEMIA; SIGNAL TRANSDUCTION; THROMBOCYTE ACTIVATION;

ANEMIA, SICKLE CELL; CELL ADHESION; ENDOTHELIUM, VASCULAR; ERYTHROCYTES; HEMOGLOBIN, SICKLE; HEMOLYSIS; HUMANS; ION CHANNELS; NITRIC OXIDE;

EID: 80455132476     PISSN: 09715916     EISSN: 09715916     Source Type: Journal    
DOI: None     Document Type: Review

References (34)

1. Pauling L, Itano H, Singer SJ, Wells IC. Sickle cell anemia: a molecular disease. Science 1949; 110: 543-8.
2. globins of normal human and sickle-cell anemia haemoglobin. Nature 1956; 178: 792-4.
3. Bunn H, Forget BG. Hemoglobin: Molecular, genetic and clinical aspects. Philadelphia, PA, USA: WB Saunders; 1986.
4. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997; 337: 762-9.
5. Hebbel RP. Adhesive interactions of sickle erythrocytes with endothelium. J Clin Invest 1997; 100 (11 Suppl): S83-6.
6. Platt OS. Sickle cell anemia as an inflammatory disease. J Clin Invest 2000; 106: 337-8.
7. Hebbel RP. Adhesion of sickle red cells to endothelium: Myths and future directions. Transfus Clin Biol 2008; 15: 14-8.
8. Chiang EY, Frenette PS. Sickle cell vaso-occlusion. Hematol Oncol Clin North Am 2005; 9: 771-94.
9. El Nemer W, Colin Y, Le Van Kim C. Role of Lu/BCAM glycoproteins in red cell diseases. Transfus Clin Biol 2010; 17: 143-7.
10. Rivera A, Jarolim P, Brugnara C. Modulation of Gardos channel activity by cytokines in sickle erythrocytes. Blood 2002; 99: 357-603.
11. Merciris P. Giraud F. How do sickle cells become dehydrated? Hematol J 2001; 2: 200-5.
12. Kato GJ, Gladwin MT, Steinberg MH. Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007; 21: 37-47.
13. Edelstein SJ, Telford JN, Crépeau RH. Structure of fibers of sickle cell hemoglobin. Proc Natl Acad Sci USA 1973; 70: 1104-7.
14. Stuart MJ, Nagel RL. Sickle-cell disease. Lancet 2004; 364: 1343-60.
15. Mohandas N, Evans E. Adherence of sickle erythrocytes to vascular endothelial cells: requirement for both cell membrane changes and plasma factors. Blood 1984; 64: 282-7.
16. Elion J, Brun M, Odievre MH, Lapoumeroulie CL, Krishnamoorthy R. Vaso-occlusion in sickle cell anemia: role of interactions between blood cells and endothelium. Hematol J 2004; 5: S195-8.
17. Solovey A, Lin Y, Browne P, Choong S, Wayner E, Hebbel RP. Circulating activated endothelial cells in sickle cell anemia. N Engl J Med 1997; 337: 1584-90.
18. Frenette PS, Atweh GF. Sickle cell disease: old discoveries, new concepts, and future promise. J Clin Invest 2007; 117: 850-8.
19. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994; 330: 1639-44.
20. Kaul DK, Hebbel RP. Hypoxia/reoxygenation causes inflammatory response in transgenic sickle mice but not in normal mice. J Clin Invest 2000; 106: 411-20.
21. Hebbel RP, Osarogiagbon R, Kaul D. The endothelial biology of sickle cell disease: inflammation and a chronic vasculopathy. Microcirculation 2004; 11: 129-51.
22. Piccin A, Murphy WG, Smith OP. Circulating microparticles: pathophysiology and clinical implications. Blood Rev 2007; 21: 157-71.
23. Merciris P, Claussen WJ, Joiner CH, Giraud F. Regulation of K-Cl cotransport by Syk and Src protein tyrosine kinases in deoxygenated sickle cells. Pflugers Arch 2003; 446: 232-8.
24. Durpès MC, Nebor D, du Mesnil PC, Mougenel D, Decastel M, Elion J, et al. Effect of interleukin-8 and RANTES on the Gardos channel activity in sickle human red blood cells: role of the Duffy antigen receptor for chemokines. Blood Cells Mol Dis 2010; 44: 219-23.
25. Hines PC, Zen Q, Burney SN, Shea DA, Ataga KI, Orringer EP, et al. Novel epinephrine and cyclic AMP-mediated activation of BCAM/Lu-dependent sickle (SS) RBC adhesion. Blood 2003; 101: 3281-7.
26. Reiter CD, Gladwin MT. An emerging role for nitric oxide in sickle cell disease vascular homeostasis and therapy. Curr Opin Hematol 2003; 10: 99-107.
27. Morris CR, Kato GJ, Poljakovic M, Wang X, Blackwelder WC, Sachdev V, et al. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension and mortality in sickle cell disease. JAMA 2005; 294: 81-90.
28. Mack AK, Kato GJ. Sickle cell disease and nitric oxide: a paradigm shift? Int J Biochem Cell Biol 2006; 38: 1237-43.
29. Bunn HF, Nathan DG, Dover GJ, Hebbel RP, Platt OS, Rosse WF, et al. Pulmonary hypertension and nitric oxide depletion in sickle cell disease. Blood 2010; 116: 687-92.
30. Hebbel RP. Reconstructing sickle cell disease: a data-based analysis of the "hyperhemolysis paradigm" for pulmonary hypertension from the perspective of evidence-based medicine. Am J Hematol 2011; 86: 123-54.
31. Hebbel RP, Vercellotti G, Nath KA. A systems biology consideration of the vasculopathy of sickle cell anemia: the need for multi-modality chemo-prophylaxsis. Cardiovasc Hematol Disord Drug Targets 2009; 9: 271-92.
32. Labie D, Elion J. Molecular and cellular pathophysiology of sickle cell anemia. Pathol Biol (Paris) 1999; 47: 7-12.
33. Elion J, Labie D. Bases physiopathologiques moléculaires et cellulaires du traitement de la drépanocytose. Hématologie 1996; 2: 499-510.
34. Elion J, Laurance S, Lapouméroulie C. Physiopathologie de la drépanocytose. Med Trop 2010; 70: 454-8.