Two cases of thrombotic thrombocytopenic purpura
| Case 1 | Case 2 | Reference range | ||
|---|---|---|---|---|
| Relapse 1 | Relapse 2 | |||
| Age | 50 | 30 | 38 | |
| White blood cell count, × 109/L | 16.09 | 14.16 | 13.39 | 3.7–11.0 |
| Hemoglobin, g/dL | 5.7 | 13.6 | 10.7 | 11.5–15.5 |
| Platelet count, × 109/L | 10 | 272 | 32 | 150–400 |
| Schistocytes | Moderate | 0 | Moderate | |
| Lactate dehydrogenase, U/L | 2,403 | 219 | 718 | 100–220 |
| Creatinine, mg/dL | 0.8 | 0.8 | 0.78 | 0.70–1.40 |
| Fibrinogen, mg/dL | 330 | 253 | 155 | 200–400 |
| Activated partial thromboplastin time, seconds | 28.7 | 29.1 | 26 | 23.0–32.4 |
| Prothrombin time, seconds | 10.9 | 10 | 10.9 | 8.4–13.0 |
| ADAMTS13 activity, % | 5 | 11 | <5–9b | ≥ 68 |
| ADAMTS13 inhibitor, BUa | 0.5 | < 0.4 | 1.8–8b | ≤ 0.4 |
| ADAMTS13 antibody, U/mL | 18 | 4 | NA | <19 |
| Treatment | Plasma exchange, prednisone | None | Plasma exchange, prednisone, dipyridamole, rituximab | |
| Follow-up | Recent ADAMTS13 activity > 114, platelets 240 | Yes | 4 months later: ADAMTS13 activity 87, platelets 474 | |