Cited by 2,340Hemoglobinopathies and Related DisordersBlood groups and transfusionHemophilia Treatment and Research

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Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia

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Charache, Samuel, Terrin, Michael L., Moore, Richard D., Dover, George J., Barton, Franca B., Eckert, Susan V., McMahon, Robert P., Bonds, Duane R.·1995·New England Journal of Medicine

DOI 10.1056/nejm199505183322001

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Abstract

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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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)
ORCID: Terrin, Michael L.
Moore, Richard D.
Barton, Franca B.
McMahon, Robert P.
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
DOI: 10.1056/nejm199505183322001
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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Effect of Hydroxyurea on the Frequency of Painful Crises in Sickle Cell Anemia

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