vWF Multimeric Pattern and vWF-Cleaving Protease in Patients With Familial HUS/TTP
| Patient (sex/age [yr]) . | Lmw vWF Multimers . | Hmw vWF Multimers . | vWF-Cleaving Protease Activity . | |||
|---|---|---|---|---|---|---|
| Acute Phase . | Remission . | Acute Phase . | Remission . | Acute Phase . | Remission . | |
| F34#1 (M/4) | — | Increased | — | Decreased | — | 100%* |
| F45#2 (F/49) | — | Increased | — | Decreased | — | 0* |
| F48#2 (F/52) | — | Increased | — | Decreased | — | 0* |
| F39#3 (F/29) | Increased | Increased | Decreased | Decreased | — | 100%* |
| F40#3 (M/37) | — | Increased | — | Decreased | — | 100%* |
| F30#4 (F/26) | — | Normal | — | Normal | — | 100%* |
| F33#4 (F/13) | — | Normal | — | Normal | — | 100%* |
| F64#5 (M/4) | — | Normal | — | Normal | — | 100% |
| F1#6 (F/42) | Increased | Increased | Decreased | Decreased | — | — |
| F101#19 (M/46) | Slightly increased | — | Slightly decreased | — | 0* | — |
| F102#19 (M/41) | — | Normal | — | Normal | — | 0* |
| F106#24 (M/24) | — | Normal | — | Normal | — | 100%* |
| F108#24 (M/22) | — | Normal | — | Normal | — | 100%* |
| F29#29a (M/1) | — | Increased | — | Decreased | — | 100% |
| F85#29b (F/1) | — | Normal | — | Normal | — | 100% |
| Patient (sex/age [yr]) . | Lmw vWF Multimers . | Hmw vWF Multimers . | vWF-Cleaving Protease Activity . | |||
|---|---|---|---|---|---|---|
| Acute Phase . | Remission . | Acute Phase . | Remission . | Acute Phase . | Remission . | |
| F34#1 (M/4) | — | Increased | — | Decreased | — | 100%* |
| F45#2 (F/49) | — | Increased | — | Decreased | — | 0* |
| F48#2 (F/52) | — | Increased | — | Decreased | — | 0* |
| F39#3 (F/29) | Increased | Increased | Decreased | Decreased | — | 100%* |
| F40#3 (M/37) | — | Increased | — | Decreased | — | 100%* |
| F30#4 (F/26) | — | Normal | — | Normal | — | 100%* |
| F33#4 (F/13) | — | Normal | — | Normal | — | 100%* |
| F64#5 (M/4) | — | Normal | — | Normal | — | 100% |
| F1#6 (F/42) | Increased | Increased | Decreased | Decreased | — | — |
| F101#19 (M/46) | Slightly increased | — | Slightly decreased | — | 0* | — |
| F102#19 (M/41) | — | Normal | — | Normal | — | 0* |
| F106#24 (M/24) | — | Normal | — | Normal | — | 100%* |
| F108#24 (M/22) | — | Normal | — | Normal | — | 100%* |
| F29#29a (M/1) | — | Increased | — | Decreased | — | 100% |
| F85#29b (F/1) | — | Normal | — | Normal | — | 100% |
Data included in study by Furlan et al.20