Clinical presentation and laboratory findings in untreated patients with acute TMA
Patient ID . | Diagnosis . | Age . | Sex . | Platelets, × 109/L . | ADAMTS13 activity, % . | Inhibitor, BU/mL . | IgG titer . | IgM titer . |
---|---|---|---|---|---|---|---|---|
P1 | Post-ticlopidine TMA | 68 | M | 14 | < 5 | Neg | 3200 | 400 |
P2 | Cancer-associated TMA | 39 | M | NA | 8 | Neg | 1600 | 100 |
P3 | Idiopathic TTP | 70 | F | 9 | < 3 | Neg | 600 | 400 |
P4 | Idiopathic TTP | 35 | F | 9 | < 6 | Neg | 300 | Neg |
P5 | Idiopathic TTP | 62 | F | NA | < 3 | Neg | 400 | Neg |
P6 | Idiopathic TTP | 56 | M | 57 | < 6 | Neg | 200 | Neg |
P7 | Idiopathic TTP | 58 | F | 92 | < 6 | 1.5 | Neg | Neg |
P8 | Idiopathic TTP | 36 | F | 13 | 14 | 2 | 20 | Neg |
P9 | Idiopathic TTP | 32 | F | 10 | 16 | ND | 1600 | Neg |
P10 | Idiopathic TTP | 17 | F | 59 | 11 | 1.5 | 3200 | Neg |
P11 | Unspecified TMA | 34 | M | NA | 35 | ND | Neg | 100 |
P12 | HUS | 23 | M | 36 | 79 | Neg | Neg | 400 |
P13 | HUS | 0.5 | M | NA | 40 | ND | Neg | 100 |
Patient ID . | Diagnosis . | Age . | Sex . | Platelets, × 109/L . | ADAMTS13 activity, % . | Inhibitor, BU/mL . | IgG titer . | IgM titer . |
---|---|---|---|---|---|---|---|---|
P1 | Post-ticlopidine TMA | 68 | M | 14 | < 5 | Neg | 3200 | 400 |
P2 | Cancer-associated TMA | 39 | M | NA | 8 | Neg | 1600 | 100 |
P3 | Idiopathic TTP | 70 | F | 9 | < 3 | Neg | 600 | 400 |
P4 | Idiopathic TTP | 35 | F | 9 | < 6 | Neg | 300 | Neg |
P5 | Idiopathic TTP | 62 | F | NA | < 3 | Neg | 400 | Neg |
P6 | Idiopathic TTP | 56 | M | 57 | < 6 | Neg | 200 | Neg |
P7 | Idiopathic TTP | 58 | F | 92 | < 6 | 1.5 | Neg | Neg |
P8 | Idiopathic TTP | 36 | F | 13 | 14 | 2 | 20 | Neg |
P9 | Idiopathic TTP | 32 | F | 10 | 16 | ND | 1600 | Neg |
P10 | Idiopathic TTP | 17 | F | 59 | 11 | 1.5 | 3200 | Neg |
P11 | Unspecified TMA | 34 | M | NA | 35 | ND | Neg | 100 |
P12 | HUS | 23 | M | 36 | 79 | Neg | Neg | 400 |
P13 | HUS | 0.5 | M | NA | 40 | ND | Neg | 100 |
Patients 1 to 7 presented with severe ADAMTS13 deficiency (< 10% of normal); discrepant results between the ADAMTS13 inhibitor assay and ELISA results for anti-ADAMTS13 IgG and IgM antibodies are shown. Patients 8 to 13 presented without severe ADAMTS13 deficiency (> 10% of normal); anti-ADAMTS13 IgG or IgM antibodies detected by ELISA are shown.
M indicates male; Neg, negative; NA, not available; F, female; and ND, not determined.