Textbook recommendations for hydroxyurea therapy in SCD
| Text . | Indications . | Initial dose, mg/kg per day . | Dose increments, amount/frequency . | Monitoring . |
|---|---|---|---|---|
| MSH, 19956 | ≥3 severe VOCs/y | 15 | 5 mg/kg per day every 12 wk | ANC >2×109/L |
| NIH, 20024 | Frequent severe VOCs* | 10-15 | Increase every 6-8 wk (increment not stated) | ANC >2.5×109/L* |
| Hematology | ||||
| Wintrobe's9 | Frequent VOCs | Not stated | Not stated | Not stated |
| Hoffman10 | “Treatment of pain crises” | 0.15 | 0.3 mg/kg per day (frequency not stated) | ANC >2×109/L |
| Williams11 | Not stated | 15 | 5 mg/kg per day every 12 wk | WBC >2×109/L† |
| Handin et al12 | Frequent pain‡ | 15 | 5 mg/kg per day every 8 wk | ANC >2×109/L |
| Bethesda13 | Frequent pain | 10-15 | 5 mg/kg per day every 6-8 wk | ANC >2×109/L§ |
| Boyiadzis et al14 | Not stated | 10-15 | 5 mg/kg per day every 12 wk | ANC >2.5×109/L |
| Young et al15 | Frequent pain∥ | Not stated | Not stated | Not stated |
| Hillman et al16 | Not stated | 10-15 or 500 mg/d | Increase to 1.0 g/d at 6-8 wk | Not stated |
| Hoffbrand et al17 | Frequent pain‡ | 15 | Up to 25 mg/kg per day | Not stated |
| Internal medicine | Not stated | |||
| Oxford21 | Not stated | Not stated | Not stated | Not stated |
| Cecil22 | Impaired by VOC | 15 | Increase to maximum tolerated | Not stated |
| Washington Manual23 | Not stated | 15-35 # | Not stated | Not stated |
| ACP Medicine24 | Not stated | 15 | Not stated | Not stated |
| Harrison's25 | ≥3 VOCs/y** | 10-30# | Not stated | WBC = 5-8×109/L |
| Conn's26 | ≥3 VOCs/y | Not stated | Not stated | Not stated |
| Up-To-Date27 | Not stated | Not stated | Not stated | Not stated |
| Text . | Indications . | Initial dose, mg/kg per day . | Dose increments, amount/frequency . | Monitoring . |
|---|---|---|---|---|
| MSH, 19956 | ≥3 severe VOCs/y | 15 | 5 mg/kg per day every 12 wk | ANC >2×109/L |
| NIH, 20024 | Frequent severe VOCs* | 10-15 | Increase every 6-8 wk (increment not stated) | ANC >2.5×109/L* |
| Hematology | ||||
| Wintrobe's9 | Frequent VOCs | Not stated | Not stated | Not stated |
| Hoffman10 | “Treatment of pain crises” | 0.15 | 0.3 mg/kg per day (frequency not stated) | ANC >2×109/L |
| Williams11 | Not stated | 15 | 5 mg/kg per day every 12 wk | WBC >2×109/L† |
| Handin et al12 | Frequent pain‡ | 15 | 5 mg/kg per day every 8 wk | ANC >2×109/L |
| Bethesda13 | Frequent pain | 10-15 | 5 mg/kg per day every 6-8 wk | ANC >2×109/L§ |
| Boyiadzis et al14 | Not stated | 10-15 | 5 mg/kg per day every 12 wk | ANC >2.5×109/L |
| Young et al15 | Frequent pain∥ | Not stated | Not stated | Not stated |
| Hillman et al16 | Not stated | 10-15 or 500 mg/d | Increase to 1.0 g/d at 6-8 wk | Not stated |
| Hoffbrand et al17 | Frequent pain‡ | 15 | Up to 25 mg/kg per day | Not stated |
| Internal medicine | Not stated | |||
| Oxford21 | Not stated | Not stated | Not stated | Not stated |
| Cecil22 | Impaired by VOC | 15 | Increase to maximum tolerated | Not stated |
| Washington Manual23 | Not stated | 15-35 # | Not stated | Not stated |
| ACP Medicine24 | Not stated | 15 | Not stated | Not stated |
| Harrison's25 | ≥3 VOCs/y** | 10-30# | Not stated | WBC = 5-8×109/L |
| Conn's26 | ≥3 VOCs/y | Not stated | Not stated | Not stated |
| Up-To-Date27 | Not stated | Not stated | Not stated | Not stated |
MSH indicates multicenter study of hydroxyurea, NIH, National Institutes of Health; VOC, vaso-occlusive crisis; ANC, absolute neutrophil count; and WBC, white blood cell count.
Other indications include acute chest syndrome, other severe vaso-occlusive events and severe anemia. Additional monitoring criteria include platelet count higher than 95×109/L.
Additional monitoring criteria include hemoglobin level more than 45 g/L (4.5 g/dL) and reticulocyte count higher than 80×109/L.
Another indication is the acute chest syndrome.
Additional monitoring criteria include reticulocyte count higher than 100×109/L and platelet count higher than 90×109/L.
Another indication is recurrent acute chest syndrome.
Only a dose range given in Washington Manual and Harrison's without details on initial dose or dose adjustment.
Severe VOC is defined as requiring hospitalization and recurrent acute chest syndrome is given as another indication for hydroxyurea treatment.