The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: Results of a 17-year, single-center trial (LaSHS)

Blood

Volumn 115, Issue 12, 2010, Pages 2354-2363

  Voskaridou, Ersi ^a   Christoulas, Dimitrios ^b   Bilalis, Antonios ^a   Plata, Eleni ^a   Varvagiannis, Konstantinos ^a   Stamatopoulos, George ^c   Sinopoulou, Klio ^a   Balassopoulou, Aggeliki ^a   Loukopoulos, Dimitris ^d   Terpos, Evangelos ^b  

^a LAIKO GENERAL HOSPITAL   (Greece)

^b UNIVERSITY OF ATHENS MEDICAL SCHOOL   (Greece)

^c G GENNIMATAS GENERAL HOSPITAL   (Greece)

^d Academy of Athens Biomedical Research Foundation   (Greece)

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN F; HYDROXYUREA; ANTISICKLING AGENT; HEMOGLOBIN S;

ADULT; AGED; ARTICLE; CANCER SURVIVAL; CLINICAL TRIAL; CONTROLLED STUDY; DRUG EFFECT; DRUG EFFICACY; DRUG SAFETY; FEMALE; HOSPITAL ADMISSION; HUMAN; MAJOR CLINICAL STUDY; MALE; NEUTROPENIA; PHASE 2 CLINICAL TRIAL; PRIORITY JOURNAL; PROBABILITY; SICKLE CELL ANEMIA; THROMBOCYTOPENIA; BLOOD TRANSFUSION; FOLLOW UP; GENETICS; HOSPITALIZATION; MIDDLE AGED; MORBIDITY; MORTALITY; MULTIVARIATE ANALYSIS; PREDICTION AND FORECASTING; PROSPECTIVE STUDY; STATISTICS; SURVIVAL; TIME;

ADULT; AGED; ANEMIA, SICKLE CELL; ANTISICKLING AGENTS; BLOOD TRANSFUSION; FEMALE; FETAL HEMOGLOBIN; FOLLOW-UP STUDIES; HEMOGLOBIN, SICKLE; HOSPITALIZATION; HUMANS; HYDROXYUREA; MALE; MIDDLE AGED; MORBIDITY; MULTIVARIATE ANALYSIS; PREDICTIVE VALUE OF TESTS; PROSPECTIVE STUDIES; SURVIVAL ANALYSIS; TIME FACTORS; YOUNG ADULT;

EID: 77950622293     PISSN: 00064971     EISSN: 15280020     Source Type: Journal    
DOI: 10.1182/blood-2009-05-221333     Document Type: Article

References (37)

1. Ballas SK, Barton FB, Waclawiw MA, et al. Hydroxyurea and sickle cell anemia: effect on quality of life. Health Qual Life Outcomes. 2006;4:59.
2. McClish DK, Penberthy LT, Bovbjerg VE, et al. Health related quality of life in sickle cell patients: the PiSCES project. Health Qual Life Outcomes. 2005;3:50.
3. Platt O, Brambilla D, Rosse W, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994;330(23):1639-1644. (Pubitemid 124005049)
4. DeSimone J, Heller P, Hall L, Zwiers D. 5-Azacytidine stimulates fetal hemoglobin synthesis in anemic baboons. Proc Natl Acad Sci U S A. 1982;79(14):4428-4431. (Pubitemid 12045807)
5. Papayannopoulou T, Torrealba de Ron A, Veith R, Knitter G, Stamatoyannopoulos G. Arabinosylcytosine induces fetal hemoglobin in baboons by perturbing erythroid cell differentiation kinetics. Science. 1984;224(4649):617-619. (Pubitemid 14137533)
6. Franco RS, Yasin Z, Palascak MB, Ciraolo P, Joiner CH, Rucknagel DL. The effect of fetal hemoglobin on the survival characteristics of sickle cells. Blood. 2006;108(3):1073-1076. (Pubitemid 44154647)
7. Platt OS, Orkin SH, Dover G, Beardsley GP, Miller B, Nathan DG. Hydroxyurea enhances fetal hemoglobin production in sickle cell anemia. J Clin Invest. 1984;74(2):652-656. (Pubitemid 14054015)
8. Charache S, Dover GJ, Moore RD, et al. Hydroxyurea: effects on hemoglobin F production in patients with sickle cell anemia. Blood. 1992;79(10):2555-2565.
9. Noguchi CT, Rodgers GP, Serjeant GR, Schechter AN. Levels of fetal hemoglobin necessary for treatment of sickle cell disease. N Engl J Med. 1988;318(2):96-99.
10. Charache S, Barton FB, Moore RD, et al. Hydroxyurea and sickle cell anemia. Clinical utility of a myelosuppressive "switching" agent. The Multicenter Study of hydroxyurea in Sickle Cell Anemia. Medicine (Baltimore). 1996;75(6):300-326. (Pubitemid 27009233)
11. Ballas SK, Marcolina MJ, Dover GJ, Barton FB. Erythropoietic activity in patients with sickle cell anemia before and after treatment with hydroxyurea. Br J Haematol. 1999;105(2):491-496. (Pubitemid 29232176)
12. King SB. Nitric oxide production from hydroxyurea. Free Radic Biol Med. 2004;37(6):737-774.
13. Cokic VP, Smith RD, Beleslin-Cokic BB, et al. Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest. 2003;111(2):231-239. (Pubitemid 36105922)
14. Voskaridou E, Kalotychou V, Loukopoulos D. Clinical and laboratory effect of long-term administration of hydroxyurea to patients with sickle cell/beta-thalassemia. Br J Haematol. 1995;89(3):479-484.
15. Loukopoulos D, Voskaridou E, Kalotychou V, Schina M, Loutradi A, Theodoropoulos I. Reduction of the clinical severity of sickle cell/betathalassemia with hydroxyurea: the experience of a single center in Greece. Blood Cells Mol Dis. 2000;26(5):453-466. (Pubitemid 32037414)
16. 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(13):1645-1651. (Pubitemid 37430269)
17. Charache S. Mechanism of action of hydroxyurea in the management of sickle cell anemia in adults. Semin Hematol. 1997;34(3 Suppl 3):15-21.
18. Morris CR, Kato GJ, Poljakovic M, et al. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. 2005;294(1):81-90. (Pubitemid 41002842)
19. Kato GJ, McGowan V, Machado RF, et al. Lactate dehydrogenase as a biomarker of hemolysisassociated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease. Blood. 2006;107(6):2279-2285. (Pubitemid 43345545)
20. Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294. (Pubitemid 28018756)
21. Traina F, Jorge SG, Yamanaka A, de Meirelles LR, Costa FF, Saad ST. Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients. Acta Haematol. 2007;118(3):129-135. (Pubitemid 350014899)
22. Stanca CM, Fiel MI, Schiano TD. Exacerbation of sickle cell disease itself as a cause of abnormal liver chemistry tests. Dig Dis Sci. 2007;52(1):176-178.
23. Ahn H, Li CS, Wang W. Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005;45(2):184-190. (Pubitemid 40993585)
24. Koskinas J, Manesis EK, Zacharakis GH, Galiatsatos N, Sevastos N, Archimandritis AJ. Liver involvement in acute vaso-occlusive crisis of sickle cell disease: prevalence and predisposing factors. Scand J Gastroenterol. 2007;42(4):499-507. (Pubitemid 46569395)
25. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325(21):1476-1481.
26. Akinyoola AL, Adediran IA, Asaleye CM, Bolarinwa AR. Risk factors for osteonecrosis of the femoral head in patients with sickle cell disease. Int Orthop. 2009;33(4):923-926.
27. Serjeant GR, Serjeant BE, Mohan JS, Clare A. Leg ulceration in sickle cell disease: medieval medicine in a modern world. Hematol Oncol Clin North Am. 2005;19(5):943-956. (Pubitemid 41427120)
28. Koshy M, Entsuah R, Koranda A, et al. Leg ulcers in patients with sickle cell disease. Blood. 1989;74(4):1403-1408. (Pubitemid 19227454)
29. Halabi-Tawil M, Lionnet F, Girot R, Bachmeyer C, Lévy PP, Aractingi S. Sickle cell leg ulcers: a frequently disabling complication and a marker of severity. Br J Dermatol. 2008;158(2):339-344.
30. Voskaridou E, Kyrtsonis MC, Loutradi-Anagnostou A. Healing of chronic leg ulcers in the hemoglobinopathies with perilesional injections of granulocyte-macrophage colony-stimulating factor. Blood. 1999;93(10):3568-3569. (Pubitemid 29220927)
31. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decate observational study of 1056 patients. Medicine (Baltimore). 2005;84(6):363-376. (Pubitemid 41668935)
32. Chaine B, Neonato MG, Girot R, Aractingi S. Cutaneous adverse reactions to hydroxyurea in patients with sickle cell disease. Arch Dermatol. 2001;137(4):467-470. (Pubitemid 32319842)
33. Shelby MD. Center for the Evaluation of Risks to Human Reproduction. NTP-CERHR Expert Panel Report on the Reproductive and Developmental toxicity of Hydroxyurea. January 2007. http://cerhr.niehs.nih.gov/chemicals/hydroxyurea/ Hydroxyurea-final.pdf. Accessed December 28, 2007.
34. Schultz WH, Ware RE. Malignancy in patients with sickle cell disease. Am J Hematol. 2003; 74(4):249-253. (Pubitemid 37484693)
35. Moschovi M, Psychou F, Menegas D, Tsangaris GT, Tzortzatou-Stathopoulou F, Nikolaidou P. Hodgkin's disease in a child with sickle cell disease treated with hydroxyurea. Pediatr Hematol Oncol. 2001;18(6):371-376. (Pubitemid 32822623)
36. Kattamis A, Lagona E, Orfanou I, et al. Clinical response and adverse events in young patients with sickle cell disease treated with hydroxyurea. Pediatr Hematol Oncol. 2004;21(4):335-342. (Pubitemid 38849687)
37. Voskaridou E, Bilalis A, Christoulas D, et al. Prolonged administration of hydroxyurea reduces morbidity and mortality in adult patients with sickle-cell syndromes: long-term experience of a single center [abstract]. Blood. 2008;112(11):515. Abstract 1445.