Indications for HSCT or hydroxyurea
| Indications to start hydroxyurea . | Historical indications for HSCT (patients ≤ 16 y) . | NIH indications for HSCT (patients ≥ 16 y) . |
|---|---|---|
| Three or more VOC requiring hospitalizations | Stroke or CNS event lasting longer than 24 hours | Irreversible end-organ damage |
| Two or more acute chest syndromes | Abnormal brain MRI | Stroke or clinically significant CNS event |
| Two or more joints with osteonecrosis | Elevated TCD | Elevated TRV ≥ 2.6 m/s |
| Symptomatic anemia | ACS with recurrent hospitalizations | Sickle-related renal insufficiency (Cr ≥ 1.5 times the upper limit of normal or biopsy proven) |
| Two or more VOC requiring hospitalizations for several years | Sickle hepatopathy (including iron overload) | |
| Osteonecrosis of multiple joints | Reversible sickle complication not ameliorated by hydroxyurea | |
| Red cell alloimmunization | Two or more VOC requiring hospitalizations for several years | |
| Sickle cell lung disease | Any ACS while on hydroxyurea |
| Indications to start hydroxyurea . | Historical indications for HSCT (patients ≤ 16 y) . | NIH indications for HSCT (patients ≥ 16 y) . |
|---|---|---|
| Three or more VOC requiring hospitalizations | Stroke or CNS event lasting longer than 24 hours | Irreversible end-organ damage |
| Two or more acute chest syndromes | Abnormal brain MRI | Stroke or clinically significant CNS event |
| Two or more joints with osteonecrosis | Elevated TCD | Elevated TRV ≥ 2.6 m/s |
| Symptomatic anemia | ACS with recurrent hospitalizations | Sickle-related renal insufficiency (Cr ≥ 1.5 times the upper limit of normal or biopsy proven) |
| Two or more VOC requiring hospitalizations for several years | Sickle hepatopathy (including iron overload) | |
| Osteonecrosis of multiple joints | Reversible sickle complication not ameliorated by hydroxyurea | |
| Red cell alloimmunization | Two or more VOC requiring hospitalizations for several years | |
| Sickle cell lung disease | Any ACS while on hydroxyurea |
ACS indicates acute chest syndrome; NIH, National Institutes of Health; MRI, magnetic resonance imaging; TCD, transcranial Doppler; TRV, tricuspid regurgitant velocity; and VOC, vaso-occlusive crises.