Table 4. ADAMTS13 activity inhibitor, kidney function, liver function, and immunological analysis of patients with very low (≤30) ADAMTS13 activity.
| Patient | Dispo. | ADAMTS-13 activity (%) [70–110] | % Inhibition ADAMTS-13 activity [<30%] a | ADAMTS-13 antibody | Clotting evidence | Schistocyte count (%) [<0.5] | Platelet (count) [150–450] | eGFR (mL/min/1.73 m 2 ) [90–120] | Albumin (g/dL) [3.4–5.4] | VWF antigen (%) [50–150] | D-dimer (µg/mL) [<0.27] | IL-6 (pg/mg) [<17] b |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Patient A | Died | 2.6 | 36.2 | Neg | No | <1 | 161.0 | 42.0 | 3.9 | 513.0 | 2.0 | 77.3 (3) |
| Patient B | Died | 7.7 | 37.2 | Neg | No | <1 | 218.0 | >120 | 4.3 | 282.0 | 20.0 | 71.4 (−2) |
| Patient C | Died | 11.9 | 37.1 | Neg | No | <1 | 102.0 | 74.0 | 2.9 | 1020.0 | 0.6 | |
| Patient D | Alive | 16.0 | 29.1 | Neg | No | 4.3 | 49.0 | 5.0 | 2.7 | 386.0 | 0.7 | 43.6 (0) |
| Patient E | Died | 16.2 | 33.6 | Neg | No | <1 | 267.0 | 43.0 | 3.5 | 442.0 | 105.0 | 12.1 (−2) |
| Patient F | Alive | 18.0 | QNS | Neg | No | 1.3 | 238.0 | 76.0 | 2.8 | 648.0 | 16.2 | 47.9 (−6) |
| Patient G | Died | 18.6 | 21.9 | Neg | No | 6.9 | 60.0 | 41.0 | 2.1 | 1325.0 | 64.6 | 7982.5 (−1) |
| Patient H | Died | 19.7 | 7.0 | Neg | Ex vivo clot | 1.5 | 485.0 | 16.0 | 2.6 | 498.0 | 7.9 | 226.0 (2) |
| Patient I | Alive | 22.6 | 4.7 | Neg | Pulmonary embolism | <1 | 264.0 | 114.0 | 2.9 | 309.0 | 2.1 | 27.1 (0) |
| Patient J | Died | 23.0 | 27.9 | Neg | No | <1 | 190.0 | 64.0 | 2.5 | 244.0 | 3.7 | 132.3 (−17) |
| Patient K | Died | 23.2 | 21.6 | Neg | No | 8.6 | 37.0 | 26.0 | 2.6 | 273.0 | 0.8 | 120.6 (−1) |
| Patient L | Alive | 23.4 | 40.5 | Neg | No | <1 | 452.0 | 101.0 | 2.7 | 264.0 | 5.4 | |
| Patient M | Died | 24.3 | 25.7 | Neg | No | <1 | 124.0 | 84.0 | 3.1 | 656.0 | 1.0 | 187.9 (0) |
| Patient N | Died | 25.4 | 32.1 | Neg | Stroke | 2.9 | 187.0 | 62.0 | 2.5 | 880.0 | 189.0 | |
| Patient O | Died | 25.6 | 23.1 | Neg | No | <1 | 197.0 | >120 | 2.8 | 598.0 | 197.6 | 1338.5 (0) |
| Patient P | Died | 26.1 | 37.0 | Neg | Arterial thrombus | <1 | 161.0 | 10.0 | 3.1 | 120.0 | 3.5 | 16.90 (0) |
| Patient Q | Alive | 26.8 | 18.2 | Neg | No | <1 | 82.0 | 44.0 | 3.4 | 78.0 | 0.8 | 25.50 (0) |
| Patient R | Died | 27.6 | 29.0 | Neg | No | 2.9 | 498.0 | 14.0 | 2.5 | 514.0 | 20.0 | 283.4 (−1) |
| Patient S | Alive | 27.7 | 33.0 | Neg | No | <1 | 190.0 | >120 | 3.3 | 262.0 | 0.5 | |
| Patient T | Died | 28.8 | 27.3 | Neg | No | <1 | 187.0 | 18.0 | 2.0 | 456.0 | 3.5 | 1826 (−12) |
| Patient U | Alive | 29.2 | 22.3 | Neg | No | 1.6 | 147.0 | 10.0 | 3.1 | 255.0 | 0.7 | 45.7 (0) |
| Patient V | Died | 30.8 | 11.8 | Neg | Ex vivo clot | 6.8 | 96.0 | 75.0 | 2.1 | 738.0 | 20.0 | 173.7 (−32) |
Abbreviations: ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; LDH, lactate dehydrogenase; Hgb, hemoglobin; eGFR, estimated glomerular filtration rate; AST, aspartate aminotransferase; VWF, Von Willebrand factor; IL-6, Interleukin 6; QNS, quantity not sufficient.
a
Missing values are due to insufficient quantity of sample.
b
Missing values are due to the test not being ordered for the patient. Number in parenthesis indicates the days relative to ADAMTS13 activity measurement.