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Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia
Open accessAbstract
BACKGROUND: In a previous open-label study of hydroxyurea therapy, the synthesis of fetal hemoglobin increased in most patients with sickle cell anemia, with only mild myelotoxicity. By inhibiting sickling, increased levels of fetal hemoglobin might decrease the frequency of painful crises. METHODS: In a double-blind, randomized clinical trial, we tested the efficacy of hydroxyurea in reducing the frequency of painful crises in adults with a history of three or more such crises per year. The trial was stopped after a mean follow-up of 21 months. RESULTS: Among 148 men and 151 women studied at 21 clinics, the 152 patients assigned to hydroxyurea treatment had lower annual rates of crises than the 147 patients given placebo (median, 2.5 vs. 4.5 crises per year, P < 0.001). The median times to the first crisis (3.0 vs. 1.5 months, P = 0.01) and the second crisis (8.8 vs. 4.6 months, P < 0.001) were longer with hydroxyurea treatment. Fewer patients assigned to hydroxyurea had chest syndrome (25 vs. 51, P < 0.001), and fewer underwent transfusions (48 vs. 73, P = 0.001). At the end of the study, the doses of hydroxyurea ranged from 0 to 35 mg per kilogram of body weight per day. Treatment with hydroxyurea did not cause any important adverse effects. CONCLUSIONS: Hydroxyurea therapy can ameliorate the clinical course of sickle cell anemia in some adults with three or more painful crises per year. Maximal tolerated doses of hydroxyurea may not be necessary to achieve a therapeutic effect. The beneficial effects of hydroxyurea do not become manifest for several months, and its use must be carefully monitored. The long-term safety of hydroxyurea in patients with sickle cell anemia is uncertain.
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“inhibition of HbS polymerization with hydroxyurea reduces the incidence of ACS (205).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [205]
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“By inducing increased levels of HbF through mechanisms that include suppressing erythropoiesis, hydroxyurea was first associated with reduction of morbidity and mortality in adults.81,82”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [81]
Experimental — accuracy not yet measuredIn the citing paper
“Administration of hydroxyurea has been shown to induce expression of HbF, which interferes with the polymerization of HbS (33).”
From Red Blood Cell Function and Dysfunction: Redox Regulation, Nitric Oxide Metabolism, Anemia (2017) · cites this paper as [33]
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“Mean ANC did not change during the study to suggest that severe myelosuppression frequently occurred that would have necessitated this decrease.”
BackgroundInfluentialMost adults with severe HbSC disease are not treated with hydroxyurea
Ghunney, W. K., Asare, E. V., Ayete-Nyampong, J. B., Oppong, S. A., Rodeghier, M., DeBaun, M. R., & Olayemi, E. (2023) · Blood Advances · doi:10.1182/bloodadvances.2022009049 · Cited by 24
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“Hydroxyurea (HU), the single FDA-approved therapeutic has good activity in young patients and beneficial effects in approximately 50% of adults [5,7-10].”
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“Initially, hydroxyurea was used to prevent pain and acute chest syndrome (Charache et al., 1995, Ferster et al., 2001, Halsey and Roberts, 2003), but studies show that reduced TCD velocity is a secondary benefit (Ghafuri et al., 2017).”
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BackgroundThe modern use of hydroxyurea for children with sickle cell anemia
Quinn, C. T., & Ware, R. E. (2025) · Haematologica · doi:10.3324/haematol.2023.284633 · Cited by 30
“Subsequently, placebo-controlled phase III trials in both adults 12 and then children 13 documented the benefits of hydroxyurea for decreasing acute vaso-occlusive painful events, transfusions, and hospital admissions.”
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CLINICAL AND HEMATOLOGICAL RESPONSES TO HYDROXYUREA IN SICILIAN PATIENTS WITH Hb S/β-THALASSEMIA
Rigano, P., Rodgers, G. P., Renda, D., Renda, M. C., Aquino, A., & Maggio, A. (2001) · Hemoglobin · doi:10.1081/hem-100103065 · Cited by 23
Vance, L. D., Rodeghier, M., Cohen, R. T., ROSEN, C. L., Kirkham, F. J., Strunk, R. C., & DeBaun, M. R. (2015) · American Journal of Hematology · doi:10.1002/ajh.23959 · Cited by 23
“However, it takes 3–6 months for hydroxyurea to take full effect [30], and as we have demonstrated, a large proportion of ACS recurrences take place during that time period.”
BackgroundMian, U. K., Tang, J., Allende, A., Heo, M., Bernstein, N., Vattappally, L., Schoenfeld, D., & Minniti, C. P. (2018) · British Journal of Haematology · doi:10.1111/bjh.15617 · Cited by 23
Fogarty, H., Gaul, A., Syed, S., Aleksejenko, N., Geoghegan, R., Conroy, H., Crampton, E., Ngwenya, N., Tuohy, E., & McMahon, C. (2021) · Irish Journal of Medical Science (1971 -) · doi:10.1007/s11845-021-02588-1 · Cited by 26
Fetal Hemoglobin Regulation in Beta-Thalassemia
Lu, H. Y., Orkin, S. H., & Sankaran, V. G. (2023) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2022.12.002 · Cited by 28
Aygün, B., Lane, A., Smart, L. R., Santos, B., Tshilolo, L. M., Williams, T. N., Olupot‐Olupot, P., Stuber, S. E., TOMLINSON, G. A., Latham, T., et al. (2024) · The Lancet Haematology · doi:10.1016/s2352-3026(24)00078-4 · Cited by 28
The acute chest syndrome of sickle cell disease
Desai, P. C., & Ataga, K. I. (2013) · Expert Opinion on Pharmacotherapy · doi:10.1517/14656566.2013.783570 · Cited by 23
Clinical Follow-Up of Hydroxyurea-Treated Adults with Sickle Cell Disease
Nzouakou, R., Bachir, D., Lavaud, A., Habibi, A., Lee, K., Lionnet, F., Lesne-Hulin, A., Jouault, H., Préhu, C., Roudot‐Thoraval, F., et al. (2010) · Acta Haematologica · doi:10.1159/000322248 · Cited by 23
Rigano, P., Pecoraro, A., Calvaruso, G., Steinberg, M. H., Iannello, S., & Maggio, A. (2013) · American Journal of Hematology · doi:10.1002/ajh.23531 · Cited by 23
Ikuta, T., Sellak, H., Odo, N., Adekile, A. D., & Gaensler, K. M. L. (2016) · PLoS ONE · doi:10.1371/journal.pone.0144561 · Cited by 23
“Because NO is a product of HU metabolism [16,17], it is plausible that the reduction in vaso-occlusive crisis frequency among patients treated with HU can be attributed to NO [18].”
BackgroundAllogeneic/Matched Related Transplantation for β-Thalassemia and Sickle Cell Anemia
Bernaudin, F., Pondarré, C., Galambrun, C., & Thuret, I. (2017) · Advances in experimental medicine and biology · doi:10.1007/978-1-4939-7299-9_4 · Cited by 26
Sarri, G., Bhor, M., Abògúnr̀in, Ṣ., Farmer, C., Nandal, S., Halloway, R., & Revicki, D. A. (2018) · Health and Quality of Life Outcomes · doi:10.1186/s12955-018-0930-y · Cited by 23
“This drug helps prevent crises in both adults and children by increasing the amount of fetal hemoglobin found in patients’ red blood cells (RBCs), thus leading to various beneficial effects on RBC structure, content, and function [8, 9].”
BackgroundImpact of early analgesia on hospitalization outcomes for sickle cell pain crisis
Payne, J. N., Aban, I., Hilliard, L., Madison, J., Bemrich‐Stolz, C. J., Howard, T. H., Brandow, A. M., Waite, E. S., & Lebensburger, J. D. (2018) · Pediatric Blood & Cancer · doi:10.1002/pbc.27420 · Cited by 23
cGMP modulation therapeutics for sickle cell disease
Conran, N., & Torres, L. S. (2019) · Experimental Biology and Medicine · doi:10.1177/1535370219827276 · Cited by 23
Masera, N., Tavecchia, L., Pozzi, L., Riva, F., Vimercati, C., Calabria, M., Ronzoni, S., Masera, G., & Perseghin, P. (2007) · Transfusion and Apheresis Science · doi:10.1016/j.transci.2007.09.005 · Cited by 23
Nur, E., van Beers, E. J., Martina, S., Cuccovillo, I., Otten, H.-M., Schnog, J. B., Meijers, J. C. M., Mantovani, A., Brandjes, D. P. M., Bottazzi, B., et al. (2011) · Blood Cells Molecules and Diseases · doi:10.1016/j.bcmd.2010.10.016 · Cited by 23
Physiologic Decline in Fetal Hemoglobin Parameters in Infants With Sickle Cell Disease
Marcus, S. J., & Ware, R. E. (1999) · Journal of Pediatric Hematology/Oncology · doi:10.1097/00043426-199909000-00013 · Cited by 23
Castro, O. L., Nouraie, M. S., & Oneal, P. A. (2014) · British Journal of Haematology · doi:10.1111/bjh.13093 · Cited by 23
“Three randomized placebo controlled trials have shown that in patients with sickle cell anaemia (SCA) hydroxycarbamide (HC) improves anaemia and lowers the frequency of pain events, chest syndrome, hospitalizations and transfusions (Charache et al, 1995; Ferster et al, 1996; Wang et al, 2011).”
BackgroundLerut, J. P., Claeys, N., Laterre, P., Lavenne-Pardonge, E., Ciccarelli, O., Cavallaro, S., Palazzo, U., Renda, D., Rigano, P., & Maggio, A. (1999) · Transplantation · doi:10.1097/00007890-199901150-00010 · Cited by 23
Pain management for sickle cell disease in children and adults
Dunlop, R., & Bennett, K. C. (2014) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd003350.pub3 · Cited by 23
RetractedThe Impact of Sickle Cell Disease on Cognitive Functioning and Learning
Bonner, M. J., Schumacher, E., Gustafson, K. E., & Thompson, R. J. (1999) · School Psychology Review · doi:10.1080/02796015.1999.12085956 · Cited by 23
Cell culture and animal models to screen for promising fetal hemoglobin-stimulating compounds
Fibach, E. (2001) · Seminars in Hematology · doi:10.1016/s0037-1963(01)90032-0 · Cited by 24
Clinical trials in sickle cell disease: Adopting the combination chemotherapy paradigm
Steinberg, M. H. (2007) · American Journal of Hematology · doi:10.1002/ajh.21033 · Cited by 24
Therapeutic Strategies for Prevention of Sickle Cell Dehydration
Brugnara, C. (2001) · Blood Cells Molecules and Diseases · doi:10.1006/bcmd.2000.0366 · Cited by 23
Blood Transfusion in Sickle Cell Disease
Davies, S. C., & Olatunji, P. O. (1995) · Vox Sanguinis · doi:10.1111/j.1423-0410.1995.tb03916.x · Cited by 23
Hydroxyurea — An Essential Medicine for Sickle Cell Disease in Africa
Luzzatto, L., & Makani, J. (2019) · New England Journal of Medicine · doi:10.1056/nejme1814706 · Cited by 24
Calvet, D., Tuilier, T., Mélé, N., Turc, G., Habibi, A., Abdallah, N. A., Majhadi, L., Hemery, F., Edjlali, M., Galacteros, F., et al. (2017) · Blood Advances · doi:10.1182/bloodadvances.2017005504 · Cited by 21
Advances in Sickle Cell Therapies in the Hydroxyurea Era
Field, J. J., & Nathan, D. G. (2014) · Molecular Medicine · doi:10.2119/molmed.2014.00187 · Cited by 24
Jain, D., Arjunan, A., Sarathi, V., Jain, H., Bhandarwar, A., Vuga, M., & Krishnamurti, L. (2016) · Pediatric Blood & Cancer · doi:10.1002/pbc.26085 · Cited by 22
Metadata
- Authors
- Charache, S., Terrin, M. L., Moore, R. D., Dover, G. J., Barton, F. B., Eckert, S. V., McMahon, R. P., & Bonds, D. R.
- Affiliations
- Johns Hopkins University (US)
- Maryland Medical Research Institute (US)
- National Heart, Lung, and Blood Institute (US)
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1995-05-18
- Volume
- 332
- Issue
- 20
- Pages
- 1317-1322
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2313541759
- Semantic Scholar
- 2c0939e2f6480b519903bb15870e5b894887e70d
- Cited by
- 2,340
- References
- 26
- Citations by year
- 2026: 67 · 2025: 97 · 2024: 77 · 2023: 88 · 2022: 95 · 2021: 92 · 2020: 80 · 2019: 78 · 2018: 85 · 2017: 104 · 2016: 114 · 2015: 101 · 2014: 82 · 2013: 79 · 2012: 73
- FWCI
- 34.43
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Bristol-Myers Squibb
- National Institutes of Health (5u01hl045696-05)
- National Heart, Lung, and Blood Institute (UO1-HL 45696, UO1 HL 45692, U01HL045692)
- National Center for Research Resources (RR00046, M01RR000046)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Health Sciences › Medicine › Hematology › Hemophilia Treatment and Research
- Keywords
- hydroxyurea, sickle cell disease, vaso-occlusive crisis, fetal hemoglobin, blood transfusion, myelosuppression, treatment efficacy, adverse effects
- MeSH terms
- Adult, Anemia, Sickle Cell, Double-Blind Method, Female, Follow-Up Studies, Humans, Hydroxyurea, Male, Pain
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