Relation of presenting features and clinical outcomes of 48 patients with clinically diagnosed idiopathic TTP-HUS to the presence or absence of severe ADAMTS13 deficiency
. | Severe ADAMTS13 deficiency (n = 16) . | Not severe ADAMTS13 deficiency (n = 32) . | P . |
|---|---|---|---|
| Presenting features | |||
| Median age, y (minimum, maximum) | 39 (19, 71) | 50 (9, 85) | .007 |
| Sex (% female) | 12 (75) | 23 (72) | 1.000 |
| Race (% African American) | 8 (50) | 8 (25) | .083 |
| Obesity (% BMI ≥30 kg/m2) | 8 (50) | 10 (31) | .206 |
| Neurologic abnormalities (%) | 8 (50) | 17 (53) | .838 |
| Acute renal failure (%) | 1 (6) | 11 (34) | .040 |
| Median platelet count, 109/L (minimum, maximum) | 11 (4, 27) | 15 (2, 95) | .085 |
| Median hematocrit, % (minimum, maximum) | 21 (15, 30) | 22 (14, 32) | .509 |
| Median LDH, U/L (minimum, maximum)* | 1524 (436, 3909) | 1107 (302, 12 587) | .094 |
| Clinical outcomes | |||
| Response (%) | 14 (88) | 24 (75) | .460 |
| Exacerbation (%)† | 8/14 (57) | 9/24 (38) | .240 |
| Median no. of plasma exchange (minimum, maximum)‡ | 20 (5, 74) | 16 (3, 68) | .429 |
| TTP-HUS-associated death (%) | 3 (19) | 7 (22) | .730 |
| All Death (%) | 3 (19) | 12 (38) | .123 |
| Relapse (%)§ | 6/14 (43) | 2/25 (8) | .016 |
. | Severe ADAMTS13 deficiency (n = 16) . | Not severe ADAMTS13 deficiency (n = 32) . | P . |
|---|---|---|---|
| Presenting features | |||
| Median age, y (minimum, maximum) | 39 (19, 71) | 50 (9, 85) | .007 |
| Sex (% female) | 12 (75) | 23 (72) | 1.000 |
| Race (% African American) | 8 (50) | 8 (25) | .083 |
| Obesity (% BMI ≥30 kg/m2) | 8 (50) | 10 (31) | .206 |
| Neurologic abnormalities (%) | 8 (50) | 17 (53) | .838 |
| Acute renal failure (%) | 1 (6) | 11 (34) | .040 |
| Median platelet count, 109/L (minimum, maximum) | 11 (4, 27) | 15 (2, 95) | .085 |
| Median hematocrit, % (minimum, maximum) | 21 (15, 30) | 22 (14, 32) | .509 |
| Median LDH, U/L (minimum, maximum)* | 1524 (436, 3909) | 1107 (302, 12 587) | .094 |
| Clinical outcomes | |||
| Response (%) | 14 (88) | 24 (75) | .460 |
| Exacerbation (%)† | 8/14 (57) | 9/24 (38) | .240 |
| Median no. of plasma exchange (minimum, maximum)‡ | 20 (5, 74) | 16 (3, 68) | .429 |
| TTP-HUS-associated death (%) | 3 (19) | 7 (22) | .730 |
| All Death (%) | 3 (19) | 12 (38) | .123 |
| Relapse (%)§ | 6/14 (43) | 2/25 (8) | .016 |
Patients with idiopathic TTP-HUS who had severe ADAMTS13 deficiency (activity < 5%) were compared with the patients with idiopathic TTP-HUS who did not have severe deficiency (ADAMTS13 activity ≥ 5%). The 2 patients with severe ADAMTS13 deficiency who had TTP-HUS associated with pregnancy are not represented in this table. Presenting features and clinical outcomes are defined in “Patients and methods.” Laboratory data are the most abnormal values on the day of diagnosis ± 7 days.
LDH values were adjusted to an upper limit of normal value of 200 U/L.
The denominator is the number of patients who achieved a response.
The number of plasma exchange treatments is given for all patients who achieved a remission.
The denominator is the number of patients who achieved a remission.