Hydroxyurea for the treatment of sickle cell anemia.

@article{Platt2008HydroxyureaFT,
  title={Hydroxyurea for the treatment of sickle cell anemia.},
  author={O. Platt},
  journal={The New England journal of medicine},
  year={2008},
  volume={358 13},
  pages={
          1362-9
        }
}
  • O. Platt
  • Published 2008
  • Medicine
  • The New England journal of medicine
An 18-year-old woman with sickle cell anemia presents with recurrent painful crises, and treatment with hydroxyurea is recommended. Hydroxyurea causes a shift toward the production of red cells containing fetal hemoglobin. A possible increase in the risk of acute leukemia due to hydroxyurea therapy remains the subject of debate. 
A predictable but life-threatening complication of hydroxyurea in a patient with sickle cell anaemia: an experience learned from a Jehovah's Witness
TLDR
This case is presented of a Jehovah's Witness with sickle cell anaemia, who developed severe bone marrow suppression due to the combined effects of hydroxyurea and CKD, resulting in a prolonged recovery period after discontinuation of hydroXYurea. Expand
Review of Medication Therapy for the Prevention of Sickle Cell Crisis.
TLDR
Clinical data concerning the uses of hydroxyurea, L-glutamine, and crizanlizumab in treating pain crises associated with sickle cell disease are reviewed. Expand
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Cutaneous manifestations of sickle cell disease including leg ulcers and acquired pseudoxanthoma elasticum are reviewed. Expand
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TLDR
Trials have shown the clinical benefit of hydroxycarbamide in a subpopulation of adult patients with SCD, with a 44% reduction in the median annual rate of painful crises, a decrease in the incidence of acute chest syndrome and an estimated 40% reduce in overall mortality over a 9-year observational period. Expand
CLINICAL AND HEMATOLOGICAL EFFECTS OF HYDROXYUREA THERAPY IN MANAGEMENT OF SICKLE CELL ANEMIA
TLDR
Hydroxyurea reduced the frequency of painful crises and diminished the number of hospitalization, transfusion, and episodes of acute chest syndrome. Expand
Decreased fetal hemoglobin over time among youth with sickle cell disease on hydroxyurea is associated with higher urgent hospital use
Hydroxyurea (HU) induces dose‐dependent increased fetal hemoglobin (HbF) for sickle cell disease (SCD). Large deviation from historical personal best (PBest) HbF, a clinic‐based version of maximumExpand
Musculoskeletal Manifestations of Sickle Cell Disease: A Review
Sickle cell disease (SCD) is an inherited disorder of abnormal haemoglobin commonly encountered in the West African sub-region. It has varied osteoarticular and non-osseous complications that mimicExpand
Clinical Follow-Up of Hydroxyurea-Treated Adults with Sickle Cell Disease
TLDR
Clinical and biological benefits of this cohort were similar to those previously reported and the risk/benefit ratio for moderate hydroxyurea doses was satisfactory, based on this relatively long retrospective study. Expand
Hydroxyurea in sickle cell disease--a study of clinico-pharmacological efficacy in the Indian haplotype.
TLDR
Hydroxyurea therapy with careful monitoring was effective in reducing the clinical severity in Indian patients who initially had higher HbF levels and the presence of ameliorating factors, such as alpha-thalassemia and the Xmn I polymorphism. Expand
Changing the Clinical Paradigm of Hydroxyurea Treatment for Sickle Cell Anemia Through Precision Medicine
TLDR
An individualized dosing model for children with SCA is developed and prospectively evaluated, designed to optimize the hydroxyurea dose and clinical response, and has resulted in HbF responses >30–40%, levels beyond what is achieved with traditional weight‐based dosing and trial and error dose escalation. Expand
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References

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Use of hydroxyurea in children ages 2 to 5 years with sickle cell disease.
TLDR
This pilot study suggests that hydroxyurea is safe and effective in young children with sickle cell disease. Expand
Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia.
TLDR
Hydroxyurea therapy can ameliorate the clinical course of sickle cell anemia in some adults with three or more painful crises per year and Maximal tolerated doses of hydroxyurea may not be necessary to achieve a therapeutic effect. Expand
Treatment of sickle cell anemia with hydroxyurea and erythropoietin.
TLDR
This study confirms that hydroxyurea therapy increases hemoglobin F production and provides objective evidence that hydroXYurea reduces the rate of hemolysis and intracellular polymerization of hemoglobin S. Expand
Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study.
TLDR
Hydroxyurea treatment had no adverse effect on height or weight gain or pubertal development in school-aged children with SCA, and the Tanner stage transitions took place at appropriate ages. Expand
Safety of hydroxyurea in children with sickle cell anemia: results of the HUG-KIDS study, a phase I/II trial. Pediatric Hydroxyurea Group.
TLDR
This Phase I/II trial shows that HU therapy is safe for children with sickle cell anemia when treatment was directed by a pediatric hematologist and no life-threatening clinical adverse events occurred and no child experienced growth failure. Expand
Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment.
TLDR
Adult patients taking hydroxyurea for frequent painful sickle cell episodes appear to have reduced mortality after 9 of years follow-up, and indications for hydroxyUREa treatment should be expanded. Expand
Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study.
TLDR
Infants with SCA tolerate prolonged hydroxyurea therapy with sustained hematologic benefits, fewer ACS events, improved growth, and possibly preserved organ function. Expand
Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea. Multicenter Study of Hydroxyurea.
TLDR
Bone marrow ability to withstand HU treatment may be important for sustained HbF increases during H U treatment of HbSS, especially in patients with high baseline neutrophil and reticulocyte counts. Expand
Hydroxyurea and sickle cell anemia: effect on quality of life
TLDR
Treatment of SS with HU improves some aspects of QOL in adult patients who already suffer from moderate-to-severe SS, and the benefit of HU treatment on QOL, other than pain scales, was limited to those patients taking HU who maintained a high HBF response, compared to those with low HbF response or on placebo. Expand
Cost‐effectiveness of hydroxyurea in sickle cell anemia
TLDR
The cost of hydroxyurea with the more intensive monitoring required when using this drug appears to be more than offset by decreased costs for medical care of painful crisis and analgesic use. Expand
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