Patient characteristics at the time of enrollment
| . | HP 1-1 (n = 129) . | HP 2-1 (n = 203) . | HP 2-2 (n = 99) . | P value . |
|---|---|---|---|---|
| Age (y) | 34 (26-44) | 32 (24-43) | 30 (23-41) | .07 |
| Female | 78 (60%) | 118 (58%) | 51 (52%) | .2 |
| Sickle cell genotype | .3 | |||
| Hb SS or Sβ0-thalassemia | 93 (72%) | 157 (77%) | 76 (77%) | |
| Hb SC | 22 (17%) | 37 (18%) | 15 (15%) | |
| Hb Sβ+-thalassemia | 14 (11%) | 9 (4%) | 8 (8%) | |
| Asthma, n (%) | 24 (19%) | 48 (24%) | 20 (20%) | .6 |
| Hydroxyurea, n (%) | 60 (47%) | 92 (45%) | 46 (46%) | 1.0 |
| Tobacco use, n (%) | 37 (29%) | 47 (23%) | 22 (22%) | .2 |
| Systolic blood pressure (mm Hg) | 120 (111-129) | 119 (110-128) | 120 (110-130) | .9 |
| Body mass index (kg/m2) | 25 (22-28) | 23 (21-27) | 24 (21-28) | .1 |
| WBC count (× 103/μL) | 9.2 (6.9-11.8) | 9.8 (7.9-11.9) | 9.8 (7.4-11.8) | .1 |
| Hemoglobin (g/dL) | 9.2 (8.2-10.7) | 9.3 (8.1-10.4) | 9.3 (8.3-10.4) | .9 |
| Hemoglobin F (%) | 5.1 (1.7-9.7) | 4.2 (1.9-8.4) | 4.3 (2.5-9.0) | .9 |
| Platelet count (× 103/μL) | 343 (253-460) | 392 (291-493) | 372 (281-487) | .04 |
| Absolute reticulocyte count (× 103/μL) | 245 (164-372) | 284 (186-393) | 272 (168-411) | .6 |
| LDH (U/L) | 309 (238-436) | 304 (231-425) | 295 (235-386) | .4 |
| Ferritin (ng/mL) | 271 (74-1056) | 393 (128-1264) | 473 (114-1202) | .5 |
| Total bilirubin (mg/dL) | 2.3 (1.3-3.9) | 2.3 (1.5-3.5) | 2.1 (1.4-3.4) | .1 |
| AST (U/L) | 35 (26-52) | 36 (27-49) | 35 (27-42) | .3 |
| ALT (U/L) | 22 (16-31) | 22 (16-31) | 23 (16-30) | .6 |
| Urine albumin concentration (mg/g creatinine) | 31 (12-163) | 36 (12-198) | 19 (8-79) | .2 |
| eGFR (mL/min/1.73 m2) | 115 (87-127) | 117 (94-129) | 123 (98-132) | .06 |
| Vaso-occlusive episodes (preceding year)∗ | 2 (1-5) | 2 (0-4) | 2 (0-4) | .2 |
| Acute chest syndrome history, n (%) | 63 (50%) | 112 (55%) | 58 (59%) | .1 |
| Stroke history, n (%) | 22 (17%) | 41 (20%) | 17 (17%) | .9 |
| Priapism history, n (%) | 11/51 (22%) | 16/85 (19%) | 11/48 (23%) | .9 |
| . | HP 1-1 (n = 129) . | HP 2-1 (n = 203) . | HP 2-2 (n = 99) . | P value . |
|---|---|---|---|---|
| Age (y) | 34 (26-44) | 32 (24-43) | 30 (23-41) | .07 |
| Female | 78 (60%) | 118 (58%) | 51 (52%) | .2 |
| Sickle cell genotype | .3 | |||
| Hb SS or Sβ0-thalassemia | 93 (72%) | 157 (77%) | 76 (77%) | |
| Hb SC | 22 (17%) | 37 (18%) | 15 (15%) | |
| Hb Sβ+-thalassemia | 14 (11%) | 9 (4%) | 8 (8%) | |
| Asthma, n (%) | 24 (19%) | 48 (24%) | 20 (20%) | .6 |
| Hydroxyurea, n (%) | 60 (47%) | 92 (45%) | 46 (46%) | 1.0 |
| Tobacco use, n (%) | 37 (29%) | 47 (23%) | 22 (22%) | .2 |
| Systolic blood pressure (mm Hg) | 120 (111-129) | 119 (110-128) | 120 (110-130) | .9 |
| Body mass index (kg/m2) | 25 (22-28) | 23 (21-27) | 24 (21-28) | .1 |
| WBC count (× 103/μL) | 9.2 (6.9-11.8) | 9.8 (7.9-11.9) | 9.8 (7.4-11.8) | .1 |
| Hemoglobin (g/dL) | 9.2 (8.2-10.7) | 9.3 (8.1-10.4) | 9.3 (8.3-10.4) | .9 |
| Hemoglobin F (%) | 5.1 (1.7-9.7) | 4.2 (1.9-8.4) | 4.3 (2.5-9.0) | .9 |
| Platelet count (× 103/μL) | 343 (253-460) | 392 (291-493) | 372 (281-487) | .04 |
| Absolute reticulocyte count (× 103/μL) | 245 (164-372) | 284 (186-393) | 272 (168-411) | .6 |
| LDH (U/L) | 309 (238-436) | 304 (231-425) | 295 (235-386) | .4 |
| Ferritin (ng/mL) | 271 (74-1056) | 393 (128-1264) | 473 (114-1202) | .5 |
| Total bilirubin (mg/dL) | 2.3 (1.3-3.9) | 2.3 (1.5-3.5) | 2.1 (1.4-3.4) | .1 |
| AST (U/L) | 35 (26-52) | 36 (27-49) | 35 (27-42) | .3 |
| ALT (U/L) | 22 (16-31) | 22 (16-31) | 23 (16-30) | .6 |
| Urine albumin concentration (mg/g creatinine) | 31 (12-163) | 36 (12-198) | 19 (8-79) | .2 |
| eGFR (mL/min/1.73 m2) | 115 (87-127) | 117 (94-129) | 123 (98-132) | .06 |
| Vaso-occlusive episodes (preceding year)∗ | 2 (1-5) | 2 (0-4) | 2 (0-4) | .2 |
| Acute chest syndrome history, n (%) | 63 (50%) | 112 (55%) | 58 (59%) | .1 |
| Stroke history, n (%) | 22 (17%) | 41 (20%) | 17 (17%) | .9 |
| Priapism history, n (%) | 11/51 (22%) | 16/85 (19%) | 11/48 (23%) | .9 |
P values < .003 are significant after the Bonferroni correction.
ALT, alanine aminotransferase; AST, aspartate aminotransferase; eGFR, estimated glomerular filtration rate; Hb, hemoglobin; WBC, white blood cell count.
Vaso-occlusive episodes were defined as acute SCD-associated pain requiring hospital admission.