Summary of the included studies focused on HRQOL with SCD following HSCT
| Author, y . | Country . | Age . | No. of participants . | Specific diagnosis . | Study type (time from HSCT) . | QOL tool . | Conditioning regimen . | GRADE . |
|---|---|---|---|---|---|---|---|---|
| Arnold et al,54 2015 | USA | Range: <21 | Patients: 16 Control: 19 | HbSS, HbSC, Sickle-β+ Thal, Sickle-β0 Thal | Cross-sectional (mean 6 y post-HSCT) | PedsQL, EQ-5D | Myeloablative (busulfan, cyclophosphamide, and rabbit antithymocyte globulin, or busulfan, fludarabine, and alemtuzumab) or reduced-intensity conditioning (melphalan, fludarabine, and alemtuzumab) | Low |
| Bhatia et al,55 2015 | USA | Mean: 8.97 Median: 7.3 Range: 2.3-20.2 | Patients: 17 Parents: 23 | HbSS, HbSC, HbS/β Thal | Longitudinal (pre-HSCT, then 180 and 365 d post-HSCT) | PedsQL 4.0 | Reduced-toxicity (busulfan, fludarabine, and alemtuzumab) | Low |
| Gallo et al,56 2019 | USA | Median: 31.2 Range: 19-52 | Patients: 11 | Not reported | Cross-sectional (median 2.6 y post-HSCT, range 1.1-4.1) | SF-36v1 | Nonmyeloablative | Moderate |
| Green et al,57 2017 | USA | Range: <22 | Patient: 18 Parent: 18 | HbSS, HbS-β0 Thal | Longitudinal (pre-HSCT, then median 3 y post-HSCT, range 1-7) | PedsQL 4.0 | Myeloablative (busulfan, fludarabine, and alemtuzumab) or reduced-intensity (fludarabine, melphalan, and alemtuzumab) | Moderate |
| Kelly et al,53 2012 | USA | Median: 8 Range: 5-18 | Patient: 7 Parent: 7 Control: 268 | Not reported | Longitudinal (pre-HSCT, then 1.5, 3, 6 and 12-mo post-HSCT) | CHRIs | Mostly myeloablative | Moderate |
| Krishnamurti et al,58 2019 | USA | Mean: 22 Median: 24 Range: 17-36 | Patient: 17 | HbSS, HbSC, S/β Thal | Longitudinal (pre-HSCT, then 100 d, 6 and 12-mo post-HSCT) | PROMIS-57 | Reduced toxicity (busulfan, fludarabine, and rabbit antithymocyte globulin) | Moderate |
| Saraf et al,59 2016 | USA | Mean: 28.6 Median: 30 Range: 17-40 | Patient: 9 | HbSS and HbSC | Longitudinal (pre-HSCT, then 30, 90 and 365 d post-HSCT) | SF-36v1 | Nonmyeloablative (alemtuzumab, low-dose irradiation) | High |
| Author, y . | Country . | Age . | No. of participants . | Specific diagnosis . | Study type (time from HSCT) . | QOL tool . | Conditioning regimen . | GRADE . |
|---|---|---|---|---|---|---|---|---|
| Arnold et al,54 2015 | USA | Range: <21 | Patients: 16 Control: 19 | HbSS, HbSC, Sickle-β+ Thal, Sickle-β0 Thal | Cross-sectional (mean 6 y post-HSCT) | PedsQL, EQ-5D | Myeloablative (busulfan, cyclophosphamide, and rabbit antithymocyte globulin, or busulfan, fludarabine, and alemtuzumab) or reduced-intensity conditioning (melphalan, fludarabine, and alemtuzumab) | Low |
| Bhatia et al,55 2015 | USA | Mean: 8.97 Median: 7.3 Range: 2.3-20.2 | Patients: 17 Parents: 23 | HbSS, HbSC, HbS/β Thal | Longitudinal (pre-HSCT, then 180 and 365 d post-HSCT) | PedsQL 4.0 | Reduced-toxicity (busulfan, fludarabine, and alemtuzumab) | Low |
| Gallo et al,56 2019 | USA | Median: 31.2 Range: 19-52 | Patients: 11 | Not reported | Cross-sectional (median 2.6 y post-HSCT, range 1.1-4.1) | SF-36v1 | Nonmyeloablative | Moderate |
| Green et al,57 2017 | USA | Range: <22 | Patient: 18 Parent: 18 | HbSS, HbS-β0 Thal | Longitudinal (pre-HSCT, then median 3 y post-HSCT, range 1-7) | PedsQL 4.0 | Myeloablative (busulfan, fludarabine, and alemtuzumab) or reduced-intensity (fludarabine, melphalan, and alemtuzumab) | Moderate |
| Kelly et al,53 2012 | USA | Median: 8 Range: 5-18 | Patient: 7 Parent: 7 Control: 268 | Not reported | Longitudinal (pre-HSCT, then 1.5, 3, 6 and 12-mo post-HSCT) | CHRIs | Mostly myeloablative | Moderate |
| Krishnamurti et al,58 2019 | USA | Mean: 22 Median: 24 Range: 17-36 | Patient: 17 | HbSS, HbSC, S/β Thal | Longitudinal (pre-HSCT, then 100 d, 6 and 12-mo post-HSCT) | PROMIS-57 | Reduced toxicity (busulfan, fludarabine, and rabbit antithymocyte globulin) | Moderate |
| Saraf et al,59 2016 | USA | Mean: 28.6 Median: 30 Range: 17-40 | Patient: 9 | HbSS and HbSC | Longitudinal (pre-HSCT, then 30, 90 and 365 d post-HSCT) | SF-36v1 | Nonmyeloablative (alemtuzumab, low-dose irradiation) | High |