Hematologic, biochemical, and cardiopulmonary effects of l-arginine supplementation or phosphodiesterase 5 inhibition in patients with sickle cell disease who are on hydroxyurea therapy

European Journal of Haematology

Volumn 82, Issue 4, 2009, Pages 315-321

  Little, Jane A ^a,b   Hauser, Kristine Partovi ^c   Martyr, Sabrina E ^a   Harris, Amy ^c   Maric, Irina ^c   Morris, Claudia R ^d   Suh, Jung H ^e   Taylor, James ^a,c   Castro, Oswaldo ^a,f   MacHado, Roberto ^a,c   Kato, Gregory ^a,c   Gladwin, Mark T ^a,c  

^a NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

^b ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^c NATIONAL INSTITUTES OF HEALTH   (United States)

^d Department of Emergency Medicine   (United States)

^e CHILDREN S HOSPITAL OAKLAND RESEARCH INSTITUTE   (United States)

^f HOWARD UNIVERSITY COLLEGE OF MEDICINE   (United States)

Author keywords

L arginine; Phosphodiesterase inhibitor; Sickle cell anemia

Indexed keywords

ARGININE; CITRULLINE; GLUTATHIONE; HEMOGLOBIN F; HYDROXYUREA; ORNITHINE; PHOSPHODIESTERASE V INHIBITOR; SILDENAFIL;

ADULT; ARTICLE; BIOCHEMISTRY; CARDIOPULMONARY EXERCISE TEST; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED STUDY; DRUG BLOOD LEVEL; DRUG EFFECT; DRUG INHIBITION; DRUG MEGADOSE; EYE DISEASE; FACE EDEMA; FEMALE; HEADACHE; HUMAN; IN VIVO STUDY; LEG EDEMA; LUNG PRESSURE; MALE; PERIORBITAL EDEMA; PRIORITY JOURNAL; RETICULOCYTE COUNT; SICKLE CELL ANEMIA; WALKING;

ADULT; ANEMIA, SICKLE CELL; ARGINASE; ARGININE; BETA-GLOBINS; CITRULLINE; CYCLIC NUCLEOTIDE PHOSPHODIESTERASES, TYPE 5; FEMALE; FETAL HEMOGLOBIN; HUMANS; HYDROXYUREA; MALE; MIDDLE AGED; ORNITHINE; PHOSPHODIESTERASE INHIBITORS; PIPERAZINES; PURINES; RETICULOCYTE COUNT; SULFONES;

EID: 61349140528     PISSN: 09024441     EISSN: 16000609     Source Type: Journal    
DOI: 10.1111/j.1600-0609.2009.01210.x     Document Type: Article

References (32)

1. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, McMahon RP, Bonds DR. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med 1995 332 : 1317 22.
2. Steinberg MH, Barton F, Castro O, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. Jama 2003 289 : 1645 51.
3. King SB. N-hydroxyurea and acyl nitroso compounds as nitroxyl (HNO) and nitric oxide (NO) donors. Curr Top Med Chem 2005 5 : 665 73.
4. Cokic VP, Smith RD, Beleslin-Cokic BB, Njoroge JM, Miller JL, Gladwin MT, Schechter AN. Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest 2003 111 : 231 9.
5. Keefer JR, Schneidereith TA, Mays A, Purvis SH, Dover GJ, Smith KD. Role of cyclic nucleotides in fetal hemoglobin induction in cultured CD34+ cells. Exp Hematol 2006 34 : 1151 61.
6. Ikuta T, Ausenda S, Cappellini MD. Mechanism for fetal globin gene expression: role of the soluble guanylate cyclase-cGMP-dependent protein kinase pathway. Proc Natl Acad Sci U S A 2001 98 : 1847 52.
7. Inoue A, Kuroyanagi Y, Terui K, Moi P, Ikuta T. Negative regulation of gamma-globin gene expression by cyclic AMP-dependent pathway in erythroid cells. Exp Hematol 2004 32 : 244 53.
8. Conran N, Fattori A, Saad ST, Costa FF. Increased levels of soluble ICAM-1 in the plasma of sickle cell patients are reversed by hydroxyurea. Am J Hematol 2004 76 : 343 7.
9. Morris CR, Kuypers FA, Larkin S, Vichinsky EP, Styles LA. Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. J Pediatr Hematol Oncol 2000 22 : 515 20.
10. Schnog JJ, Jager EH, van der Dijs FP, Duits AJ, Moshage H, Muskiet FD, Muskiet FA. Evidence for a metabolic shift of arginine metabolism in sickle cell disease. Ann Hematol 2004 83 : 371 5.
11. Morris CR, Kato GJ, Poljakovic M, Wang X, Blackwelder WC, Sachdev V, Hazen SL, Vichinsky EP, Morris SM Jr., Gladwin MT. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. Jama 2005 294 : 81 90.
12. Morris CR, Morris SM, Hagar W, Van Warmerdam J, Claster S, Kepka-Lenhart D, Machado L, Kuypers FA, Vichinsky EP. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 2003 168 : 63 9.
13. Romero JR, Suzuka SM, Nagel RL, Fabry ME. Arginine supplementation of sickle transgenic mice reduces red cell density and Gardos channel activity. Blood 2002 99 : 1103 8.
14. Dasgupta T, Hebbel RP, Kaul DK. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 2006 41 : 1771 80.
15. Bialecki ES, Bridges KR. Sildenafil relieves priapism in patients with sickle cell disease. Am J Med 2002 113 : 252.
16. Burnett AL, Bivalacqua TJ, Champion HC, Musicki B. Feasibility of the use of phosphodiesterase type 5 inhibitors in a pharmacologic prevention program for recurrent priapism. J Sex Med 2006 3 : 1077 84.
17. Morris SM Jr., Kepka-Lenhart D, Chen LC. Differential regulation of arginases and inducible nitric oxide synthase in murine macrophage cells. Am J Physiol 1998 275 (5 Pt 1 E740 7.
18. Little JA, McGowan VR, Kato GJ, et al. Combination erythropoietin- hydroxyurea therapy in sickle cell disease: experience from the National Institutes of Health and a literature review. Haematologica 2006 91 : 1076 83.
19. Morris CR, Suh JH, Hagar W, Larkin S, Bland DA, Steinberg MH, Vichinsky EP, Shigenaga M, Ames B, Kuypers FA, Klings ES. Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease. Blood 2008 111 : 402 10.
20. Anthi A, Machado RF, Jison ML, et al. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J Respir Crit Care Med 2007 175 : 1272 9.
21. Dover GJ, Smith KD, Chang YC, Purvis S, Mays A, Meyers DA, Sheils C, Serjeant G. Fetal hemoglobin levels in sickle cell disease and normal individuals are partially controlled by an X-linked gene located at Xp22.2. Blood 1992 80 : 816 24.
22. Maier-Redelsperger M, de Montalembert M, Flahault A, Neonato MG, Ducrocq R, Masson MP, Girot R, Elio J. Fetal hemoglobin and F-cell responses to long-term hydroxyurea treatment in young sickle cell patients. The French Study Group on Sickle Cell Disease. Blood 1998 91 : 4472 9.
23. Wyszynski DF, Baldwin CT, Cleves MA, Amirault Y, Nolan VG, Farrell JJ, Bisbee A, Kutlar A, Farrer LA, Steinberg MH. Polymorphisms near a chromosome 6q QTL area are associated with modulation of fetal hemoglobin levels in sickle cell anemia. Cell Mol Biol (Noisy-le-grand) 2004 50 : 23 33.
24. Ma Q, Wyszynski DF, Farrell JJ, Kutlar A, Farrer LA, Baldwin CT, Steinberg MH. Fetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea. Pharmacogenomics J 2007 7 : 386 94.
25. Bender AT, Beavo JA. Cyclic nucleotide phosphodiesterases: molecular regulation to clinical use. Pharmacol Rev 2006 58 : 488 520.
26. Spector EB, Rice SC, Kern RM, Hendrickson R, Cederbaum SD. Comparison of arginase activity in red blood cells of lower mammals, primates, and man: evolution to high activity in primates. Am J Hum Genet 1985 37 : 1138 45.
27. Morris CR, Kuypers FA, Kato GJ, Lavrisha L, Larkin S, Singer T, Vichinsky EP. Hemolysis-associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci 2005 1054 : 481 5.
28. Morris CR, Vichinsky EP, van Warmerdam J, Machado L, Kepka-Lenhart D, Morris SM Jr., Kuypers FA. Hydroxyurea and arginine therapy: impact on nitric oxide production in sickle cell disease. J Pediatr Hematol Oncol 2003 25 : 629 34.
29. Griffith OW. Biologic and pharmacologic regulation of mammalian glutathione synthesis. Free Radic Biol Med 1999 27 : 922 35.
30. Wu G. Intestinal mucosal amino acid catabolism. J Nutr 1998 128 : 1249 52.
31. Machado RF, Martyr S, Kato GJ, Barst RJ, Anthi A, Robinson MR, et al. Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol 2005 130 : 445 53.
32. Styles L, Kuypers F, Kesler K, Reiss U, Lebeau P, Nagel R, et al. Arginine therapy does not benefit children with sickle cell anemia - Results of the CSCC Clinical Trial Consortium Multi-institutional Study. Blood 2007 : Abstract #2252.