Sickle cell patient characteristics and laboratory values
Patient no. . | Age, y/sex . | Genotype . | Hydroxyurea . | Leukocytes, 103/μL . | Platelets, 103/μL . | Reticulocytes, 106/μL . | Hematocrit, % . | LDH, units/L . | ADAMTS13,* % . | ||
---|---|---|---|---|---|---|---|---|---|---|---|
Activity . | Antigen . | Specific activity . | |||||||||
1 | 50/F | SS | Yes | 6.62 | 284 | 175 | 29 | 381 | — | — | — |
5.23 | 228 | 125 | 28 | 225 | 60 | 72 | 84 | ||||
10.52 | 326 | 82 | 25 | 342 | 94 | 78 | 120 | ||||
2 | 34/M | SS | Yes† | 17.94 | 576 | 205 | 25 | 465 | 63 | 115 | 55 |
17.40 | 653 | 170 | 25 | 491 | 137 | 119 | 115 | ||||
3 | 45/M | SS | Yes | 6.79 | 554 | 311 | 24 | 282 | |||
8.71 | 317 | 211 | 29 | 514 | 99 | 99 | 100 | ||||
4 | 49/M | SS | Yes | 5.48 | 150 | 244 | 25 | 394 | 112 | 116 | 97 |
5 | 25/M | SS | Yes† | 12.74 | 359 | 259 | 26 | 210 | 86 | 113 | 76 |
6 | 26/F | SS | No | 9.92 | 368 | 294 | 26 | 485 | 106 | 108 | 98 |
7 | 23/F | SS | No | 16.26 | 413 | 447 | 23 | 378 | 87 | 112 | 78 |
8 | 48/M | SS | Yes† | 11.21 | 323 | 271 | 23 | 418 | 100 | 132 | 76 |
9 | 24/F | SS | Yes | 7.16 | 366 | 313 | 28 | 250 | 94 | 103 | 91 |
10 | 29/M | Sβ0 | Yes | 9.44 | 310 | 270 | 31 | 395 | 104 | 108 | 96 |
11.00 | 464 | 280 | 34 | 437 | 144 | 119 | 120 | ||||
11 | 24/M | SC | Yes† | 6.58 | 426 | 124 | 33 | 215 | 198 | 139 | 142 |
6.95 | 388 | 111 | 30 | 210 | 159 | 124 | 128 | ||||
12 | 19/F | Sβ+ | No | 11.66 | 443 | 326 | 22 | 235 | 113 | 119 | 95 |
13 | 21/F | Sβ+ | No | 4.84 | 401 | 192 | 33 | 220 | 110 | 128 | 86 |
Normal range | 4.3-10 | 150-400 | 20-65 | 36-45 | 80-190 | 100 | 100 | 100 |
Patient no. . | Age, y/sex . | Genotype . | Hydroxyurea . | Leukocytes, 103/μL . | Platelets, 103/μL . | Reticulocytes, 106/μL . | Hematocrit, % . | LDH, units/L . | ADAMTS13,* % . | ||
---|---|---|---|---|---|---|---|---|---|---|---|
Activity . | Antigen . | Specific activity . | |||||||||
1 | 50/F | SS | Yes | 6.62 | 284 | 175 | 29 | 381 | — | — | — |
5.23 | 228 | 125 | 28 | 225 | 60 | 72 | 84 | ||||
10.52 | 326 | 82 | 25 | 342 | 94 | 78 | 120 | ||||
2 | 34/M | SS | Yes† | 17.94 | 576 | 205 | 25 | 465 | 63 | 115 | 55 |
17.40 | 653 | 170 | 25 | 491 | 137 | 119 | 115 | ||||
3 | 45/M | SS | Yes | 6.79 | 554 | 311 | 24 | 282 | |||
8.71 | 317 | 211 | 29 | 514 | 99 | 99 | 100 | ||||
4 | 49/M | SS | Yes | 5.48 | 150 | 244 | 25 | 394 | 112 | 116 | 97 |
5 | 25/M | SS | Yes† | 12.74 | 359 | 259 | 26 | 210 | 86 | 113 | 76 |
6 | 26/F | SS | No | 9.92 | 368 | 294 | 26 | 485 | 106 | 108 | 98 |
7 | 23/F | SS | No | 16.26 | 413 | 447 | 23 | 378 | 87 | 112 | 78 |
8 | 48/M | SS | Yes† | 11.21 | 323 | 271 | 23 | 418 | 100 | 132 | 76 |
9 | 24/F | SS | Yes | 7.16 | 366 | 313 | 28 | 250 | 94 | 103 | 91 |
10 | 29/M | Sβ0 | Yes | 9.44 | 310 | 270 | 31 | 395 | 104 | 108 | 96 |
11.00 | 464 | 280 | 34 | 437 | 144 | 119 | 120 | ||||
11 | 24/M | SC | Yes† | 6.58 | 426 | 124 | 33 | 215 | 198 | 139 | 142 |
6.95 | 388 | 111 | 30 | 210 | 159 | 124 | 128 | ||||
12 | 19/F | Sβ+ | No | 11.66 | 443 | 326 | 22 | 235 | 113 | 119 | 95 |
13 | 21/F | Sβ+ | No | 4.84 | 401 | 192 | 33 | 220 | 110 | 128 | 86 |
Normal range | 4.3-10 | 150-400 | 20-65 | 36-45 | 80-190 | 100 | 100 | 100 |
Patients 1,2,3,10, and 11 were tested more than once.
LDH indicates lactate dehydrogenase in plasma; SS, homozygous sickle disease; SC, hemoglobin SC disease; Sβ+, sickle β (+) thalassemia; and Sβ0, sickle β (0) thalassemia.
Values were expressed relative to pooled normal plasma.
Patient reported intermittent noncompliance.