Cochrane · systematic review · CD002202
Hydroxyurea (hydroxycarbamide) for sickle cell disease
Rankine-Mullings, AE, Nevitt, SJ · Cochrane Database of Systematic Reviews · 2022 · Cochrane Library
SoF tableOnly the outcomes this review put in its Summary of Findings table are listed (the Cochrane Handbook caps them at about seven per comparison). Its other analyses are not shown here.
Questions
28
current version
GRADE rated
22
No studies
6
Included studies
NR
No studies 6 · 21.4%Ungraded 0 · 0%Very low 9 · 32.1%Low 6 · 21.4%Moderate 7 · 25%High 0 · 0%
Ungraded means an effect was reported without a GRADE rating; it is not a gap. No studies means the review found nothing to answer the question. The six categories are a display order, not a score.
Questions and results
Current version onlySummary of findings ‐ Hydroxyurea compared with placebo for sickle cell disease
Population: adults and children with sickle cell disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alterationa Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 5 studies · 784 participants | Moderate Downgraded: imprecision, inconsistency, indirectness |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 4 studies · 759 participants | Moderate Downgraded: imprecision, inconsistency, indirectness |
Death during the study (all deaths) Summary of findings ‐ Hydroxyurea vs placebo | RR 0.65 [0.19, 2.27] | 5 studies · 784 participants | Moderate Downgraded: imprecision, inconsistency, indirectness |
Measures of HbF (%) Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 5 studies · 784 participants | Low Downgraded: imprecision, inconsistency, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 4 studies · 724 participants | Moderate Downgraded: imprecision, inconsistency, indirectness |
Quality of life: 'Health Status Survey', the 'Profile of Mood States' and the 'Ladder of Life' Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 1 study · 277 participants | Low Downgraded: imprecision, inconsistency, indirectness |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 5 studies · 784 participants | Low Downgraded: imprecision, inconsistency, indirectness |
Summary of findings ‐ Hydroxyurea and phlebotomy compared to transfusion and chelation for people with sickle cell disease and an increased risk of stroke
Population: adults and children with sickle cell disease and an increased risk of stroke
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration: proportion experiencing serious VOC or sickle‐related pain events Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | RR 3.37 [1.59, 7.11] | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Life‐threatening illness Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Death during the study (all deaths) Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | RR 0.99 [0.06, 15.42] | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Measures of HbF (%) Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Quality of life Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | NR | No studies |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Summary of findings ‐ Hydroxyurea compared with observation for people with sickle cell disease and an increased risk of stroke
Population: adults and children with sickle cell disease and an increased risk of stroke
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | NR | No studies |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Death during the study Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Measures of HbF Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Quality of life Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | NR | No studies |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Summary of findings ‐ Hydroxyurea compared with no hydroxyurea for people with sickle cell disease
Population: adults and children with sickle cell disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Death during the study Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Measures of HbF Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Quality of life Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Adverse events or toxicity Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Summary of findings ‐ Hydroxyurea compared with placebo for sickle cell disease
Population: adults and children with sickle cell disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration1 Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 4 studies · 577 participants | Moderate Downgraded: imprecision, indirectness |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 3 studies · 552 participants | Moderate Downgraded: imprecision, indirectness |
Death during the study (all deaths) Summary of findings ‐ Hydroxyurea vs placebo | RR 0.39 [0.08, 1.96] | 4 studies · 577 participants | Moderate Downgraded: imprecision, indirectness |
Measures of HbF (%) Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 4 studies · 577 participants | Moderate Downgraded: imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 3 studies · 517 participants | Moderate Downgraded: imprecision, indirectness |
Quality of life: 'Health Status Survey' the 'Profile of Mood States' and the 'Ladder of Life' Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 1 study · 277 participants | Low Downgraded: imprecision, indirectness |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea vs placebo | no estimate extracted | 4 studies · 577 participants | Low Downgraded: imprecision, indirectness |
Summary of findings ‐ Hydroxyurea and phlebotomy compared to transfusion and chelation for people with sickle cell disease and an increased risk of stroke
Population: adults and children with sickle cell disease and an increased risk of stroke
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration: proportion experiencing serious VOC or sickle‐related pain events Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | RR 3.37 [1.59, 7.11] | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Life‐threatening illness Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Death during the study (all deaths) Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | RR 0.99 [0.06, 15.42] | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Measures of HbF (%) Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Moderate Downgraded: imprecision, indirectness |
Quality of life Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 3 participants | Ungraded |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea and phlebotomy vs transfusion and chelation | no estimate extracted | 2 studies · 254 participants | Low Downgraded: imprecision, indirectness |
Summary of findings ‐ Hydroxyurea compared with observation for people with sickle cell disease and an increased risk of stroke
Population: adults and children with sickle cell disease and an increased risk of stroke
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | NR | No studies |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Death during the study Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Measures of HbF Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Quality of Life Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 2 participants | Ungraded |
Adverse events or toxicity: differences in rates of specific adverse events Summary of findings ‐ Hydroxyurea vs observation | no estimate extracted | 1 study · 22 participants | Very low Downgraded: imprecision, indirectness |
Summary of findings ‐ Hydroxyurea compared with no hydroxyurea for people with sickle cell disease
Population: adults and children with sickle cell disease
| Outcome | Effect | Evidence | Status / GRADE |
|---|---|---|---|
Pain alteration Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Life‐threatening illness Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Death during the study Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Measures of HbF Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Measures of ANC Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |
Quality of life Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | NR | No studies |
Adverse events or toxicity Summary of findings ‐ Hydroxyurea vs no hydroxyurea | no estimate extracted | 1 study · 44 participants | Very low Downgraded: risk of bias, imprecision, indirectness |