Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia: Risks and Benefits Up to 9 Years of Treatment

JAMA

Volumn 289, Issue 13, 2003, Pages 1645-1651

  Steinberg, Martin H ^a,b,c   Barton, Franca ^d   Castro, Oswaldo ^e   Pegelow, Charles H ^f   Ballas, Samir K ^g   Kutlar, Abdullah ^h   Orringer, Eugene ⁱ   Bellevue, Rita ^j   Olivieri, Nancy ^k   Eckman, James ^l   Varma, Mala ^m   Ramirez, Gloria ⁿ   Adler, Brian ^o   Smith, Wally ^p   Carlos, Timothy ^q   Ataga, Kenneth ⁱ   DeCastro, Laura ^r   Bigelow, Carolyn ^c   Saunthararajah, Yogen ^s   Telfer, Margaret ^t   Vichinsky, Elliott ^u   Claster, Susan ^v   Shurin, Susan ^w   Bridges, Kenneth ^y   Waclawiw, Myron ^x   Bonds, Duane ^x   Terrin, Michael ^d   more..

^a BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b BOSTON MEDICAL CENTER   (United States)

^c UNIVERSITY OF MISSISSIPPI SCHOOL OF MEDICINE   (United States)

^d MARYLAND MEDICAL RESEARCH INSTITUTE   (United States)

^e HOWARD UNIVERSITY COLLEGE OF MEDICINE   (United States)

^f UNIVERSITY OF MIAMI MILLER SCHOOL OF MEDICINE   (United States)

^g THOMAS JEFFERSON UNIVERSITY   (United States)

^h MEDICAL COLLEGE OF GEORGIA   (United States)

ⁱ UNIVERSITY OF NORTH CAROLINA   (United States)

^j NEW YORK METHODIST HOSPITAL   (United States)

^k HOSPITAL FOR SICK CHILDREN   (Canada)

^l EMORY UNIVERSITY   (United States)

^m UNIVERSITY OF MEDICINE AND DENTISTRY OF NEW JERSEY   (United States)

ⁿ Roosevelt Medical Center   (United States)

^o UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^p VIRGINIA COMMONWEALTH UNIVERSITY   (United States)

^q UNIVERSITY OF PITTSBURGH   (United States)

^r DUKE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^s UNIVERSITY OF ILLINOIS AT CHICAGO   (United States)

^t MICHAEL REESE HOSPITAL   (United States)

^u CHILDREN S HOSPITAL OAKLAND   (United States)

^v UNIVERSITY OF CALIFORNIA   (United States)

^w CASE WESTERN RESERVE UNIVERSITY   (United States)

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

^y NONE

more..

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN F; HYDROXYUREA; PLACEBO; ANTISICKLING AGENT;

ACUTE CHEST SYNDROME; ADULT; ARTICLE; BLOOD CELL COUNT; CANADA; CANCER; CAUSE OF DEATH; CEREBROVASCULAR DISEASE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DOUBLE BLIND PROCEDURE; FOLLOW UP; GASTROINTESTINAL DISEASE; HUMAN; KIDNEY FAILURE; LIVER DISEASE; LUNG DISEASE; MAJOR CLINICAL STUDY; MORBIDITY; MORTALITY; MULTICENTER STUDY; PRIORITY JOURNAL; RANDOMIZED CONTROLLED TRIAL; RISK BENEFIT ANALYSIS; SEPSIS; SICKLE CELL ANEMIA; STROKE; UNITED STATES; VASCULAR DISEASE; METABOLISM; PATHOPHYSIOLOGY; RISK ASSESSMENT; SURVIVAL;

ADULT; ANEMIA, SICKLE CELL; ANTISICKLING AGENTS; BLOOD CELL COUNT; CAUSE OF DEATH; DOUBLE-BLIND METHOD; FETAL HEMOGLOBIN; FOLLOW-UP STUDIES; HUMANS; HYDROXYUREA; MORBIDITY; RISK ASSESSMENT; SURVIVAL ANALYSIS;

EID: 0037414164     PISSN: 00987484     EISSN: None     Source Type: Journal    
DOI: 10.1001/jama.289.13.1645     Document Type: Article

References (33)

1. Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994;330:1639-1644.
2. Charache S, Terrin ML, Moore RD, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. N Engl J Med. 1995;332:1317-1322.
3. Charache S, Barton FB, Moore RD, et al. Hydroxyurea and sickle cell anemia. Medicine (Baltimore). 1996;75:300-326.
4. Ballas SK, Marcolina MJ, Dover GJ, Barton FB. Erythropoietic activity in patients with sickle cell anaemia before and after treatment with hydroxyurea. Br J Haematol. 1999;105:491-496.
5. Charache S, Terrin ML, Moore RD, et al. Design of the multicenter study of hydroxyurea in sickle cell anemia. Control Clin Trials. 1995;16:432-446.
6. Steinberg MH, Lu Z-H, Barton FB, et al. Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Blood. 1997;89:1078-1088.
7. Feinberg SE. The Analysis of Cross-Classified Categorical Data. 2nd ed. Cambridge, Mass: The MIT Press; 1980.
8. Draper NR, Smith H. Applied Regression Analysis. New York, NY: John Wiley; 1966.
9. Liang KY, Zeger SL. Longitudinal data analysis using generalized linear models. Biometrika. 1986;73: 13-22.
10. Kaplan EL, Meier P. Non parametric estimation from incomplete observations. J Am Stat Assoc. 1958; 53:457-481.
11. Steinberg MH, Nagel RL, Brugnara C. Cellular effects of hydroxyurea in Hb SC disease. Br J Haematol. 1997;98:838-844.
12. Ferster A, Tahriri P, Vermylen C, et al. Five years of experience with hydroxyurea in children and young adults with sickle cell disease. Blood. 2001;97:3628-3632.
13. Vichinsky EP, Neumayr LD, Eades AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. N Engl J Med. 2000;342:1855-1865.
14. Vichinsky EP, Styles LA, Colangelo LH, et al. Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood. 1997;89:1787-1792.
15. Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. Blood. 1994;84:643-649.
16. Platt OS, Thorington BD, Brambilia DJ, et al. Pain in sickle cell disease-rates and risk factors. N Engl J Med. 1991;325:11-16.
17. Bridges KR, Barabino GD, Brugnara C, et al. A multiparameter analysis of sickle erythrocytes in patients undergoing hydroxyurea therapy. Blood. 1996;88: 4701-4710.
18. Kostis JB, Turkevich D, Sharp J. Association between leukocyte count and the presence and extent of coronary atherosclerosis as determined by coronary arteriography. Am J Cardiol. 1984;53:997-999.
19. Friedman GD, Klatsky AL, Siegelaub AB. Predictors of sudden cardiac death. Circulation. 1975;52 (suppl 6):III164-III169.
20. Powars DR, Elliott Mills DD, Chan L. Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality. Ann Intern Med. 1991;115; 614-620.
21. Weinfeld A, Swolin B, Westin J. Acute leukaemia after hydroxyurea therapy in polycythemia vera and allied disorders. Eur J Haematol. 1994;52:134-139.
22. Nand S, Stock W, Godwin J, Fisher SG. Leukemogenic risk of hydroxyurea therapy in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Am J Hematol. 1996; 52:42-46.
23. Najean Y, Rain JD. Treatment of polycythemia vera: the use of hydroxyurea and pipobroman in 292 patients under the age of 65 years. Blood. 1997;90: 3370-3377.
24. 32P alone or in combination with maintenance therapy using hydroxyurea in 461 patients greater than 65 years of age. Blood. 1997;89:2319-2327.
25. Fenaux P, Preudhomme C, Laï JL, Sterkers Y. Is hydroxyurea leukemogenic in essential thrombocythemia? Blood. 1998;92:1460-1461.
26. Sterkers Y, Preudhomme C, Laï JL, et al. Acute myeloid leukemia and myelodysplastic syndromes following essential thrombocythemia treated with hydroxyurea. Blood. 1998;91:616-622.
27. Liozon E, Brigaudeau C, Trimoreau F, et al. Is treatment with hydroxyurea leukemogenic in patients with essential thrombocythemia? Hematol Cell Ther. 1997; 39:11-18.
28. Barbui T, Finazzi G, Ruggeri M, Rideghiero F. Secondary leukemia following hydroxyurea in patients with essential thrombocythemia. Blood. 1998;92:489.
29. Wilson S. Acute leukemia in a patient with sickle-cell anemia treated with hydroxyurea. Ann Intern Med. 2000;133:925-926.
30. Rauch A, Borromeo M, Ghafoor A. Leukemogenesis of hydroxyurea in the treatment of sickle cell anemia. Blood. 1999;94:415.
31. Al Jam'a AH, Al Dabbous IA, Al Khatti AA, Esan FG. Are we underestimating the leukemogenic risk of hydroxyurea. Saudi Med J. 2002;23:1411-1413.
32. Anson JA, Koshy M, Ferguson L, Crowell RM. Subarachnoid hemorrhage in sickle-cell disease. J Neurosurg. 1991;75:552-558.
33. Ohene-Frempong K, Weiner SJ, Steeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91:288-294.