Table1:
Demographic features and markers of hemolysis in the UIC and Walk-PHaSST cohorts.
| UIC | Walk-PHaSST | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Demographics | ||||||||||
| N | Females | N | Males | P-value | N | Females | N | Males | P-value | |
| Age | 224 | 34 (25–45) | 146 | 28 (22–41) | 0.016 | 364 | 36 (25–47) | 304 | 33 (22–45) | 0.005 |
| HU Use | 224 | 98 (44%) | 146 | 66 (45%) | 0.8 | 364 | 115 (32%) | 304 | 105 (35%) | 0.4 |
| - SS/SB0 | 161 (72%) | 119 (82%) | 246 (73%) | 225 (78%) | ||||||
| ALT (U/L) | 223 | 22 (15 – 32) | 146 | 23 (17 – 31) | 0.6 | - | - | - | - | - |
| α-thalassemia | 220 | 80 (37%) | 137 | 41 (30%) | 0.3 | 302 | 99 (33%) | 259 | 83 (32%) | 0.9 |
| G6PD A- | 217 | 8 (4%) | 143 | 16 (11%) | 0.005 | 263 | 10 (4%) | 222 | 23 (10%) | 0.004 |
| HbF % | 188 | 4.0 (1.8–7.6) | 118 | 3.1 (1.3–6.1) | 0.008 | 182 | 3.0 (1.1–7.2) | 155 | 2.3 (1–5.5) | 0.003 |
| Markers of Hemolysis | ||||||||||
| AST (U/L) | 218 | 34 (25–47) | 145 | 39 (30–52) | 0.04 | 319 | 35 (25–49) | 280 | 41 (30–60) | <0.0001 |
| Hemoglobin (g/dL) | 224 | 8.9 (8.0 – 10.2) | 146 | 9.1 (8.1 – 10.6) | 0.2 | 321 | 9.1 (8.0 – 10.4) | 287 | 9.4 (8.2 – 11.0) | < 0.0001 |
| LDH (U/L) | 203 | 287 (212–396) | 132 | 342 (260–494) | <0.0001 | 305 | 351 (239–494) | 260 | 396 (277–636) | 0.01 |
| Retic count (x103 U/L) | 222 | 256 (171–373) | 146 | 333 (219–441) | 0.03 | 303 | 187 (127–281) | 272 | 232 (157–321) | 0.007 |
| Total Bilirubin (mg/dL) | 217 | 1.9 (1.3–3.2) | 145 | 2.5 (1.8–4.0) | 0.002 | 325 | 3.4 (2.1–5.5) | 285 | 4.4 (2.7–6.9) | 0.0005 |
| Hemolysis-Related Complications | ||||||||||
| CKD | 196 | 102 (52%) | 121 | 61 (50%) | 0.7 | 201 | 101 (50%) | 161 | 84 (52%) | 0.2 |
| Leg ulcers | 217 | 14 (6%) | 142 | 26 (18%) | 0.0004 | 364 | 65 (18%) | 304 | 61(20%) | 0.3 |
| Pulse pressure >60mmhg | 224 | 28 (13%) | 146 | 44 (30%) | <0.0001 | 334 | 57 (17%) | 289 | 79 (27%) | 0.0004 |
| Stroke | 224 | 48 (21%) | 146 | 29 (20%) | 0.4 | 334 | 22 (7%) | 289 | 28 (10%) | 0.2 |
| TRJV >3.0 m/s | 146 | 6 (4%) | 94 | 9 (10%) | 0.1 | 312 | 30 (10%) | 273 | 43 (16%) | 0.004 |
| Vaso-occlusive Complications | ||||||||||
| ACS | 186 | 117 (63%) | 118 | 64 (54%) | 0.2 | 364 | 192 (53%) | 304 | 188 (62%) | 0.05 |
| AVN | 186 | 70 (38%) | 118 | 42 (36%) | 0.8 | 364 | 113 (31%) | 304 | 88 (29%) | 0.7 |
| VOC ≥ 3/year | 163 | 95 (58%) | 108 | 67 (62%) | 0.5 | 364 | 143 (39%) | 304 | 106 (35%) | 0.06 |
Continuous variables are presented as median values (interquartile range). UIC: University of Illinois at Chicago; Walk-PHaSST: Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy; HU: Hydroxyurea; G6PD: Glucose −6-Phosphate Dehydrogenase; HbF: Hemoglobin F; LDH: Lactate Dehydrogenase; AST: Aspartate Aminotransferase; CKD: Chronic Kidney Disease; TRJV: Tricuspid Regurgitant Jet Velocity; ACS: Acute Chest Syndrome; AVN: Avascular Necrosis; VOC: Vaso-Occlusive Crises