Table 1.
Laboratory values at different timepoints of the disease.
| Patients, stage | Year | Acute (A) or | Platelets | ADAMTS13 | IgG-ADAMTS13 | BU/mL | ADAMTS13 antigen° |
|---|---|---|---|---|---|---|---|
| Remission | ×109/L (range)° | activity % | inhibitor UI/mL | UI/mL | |||
| (R)* | (n.v.65-130) | (n.v.<17) | (n.v.0.6-1.6) | ||||
| Patient 1 | 1996 | A | 10 | <5 | N.A. | >1 | NA |
| Pre-splenectomy | 2000 | R | 287 | <5 | >120 | NA | 0.50 |
| 2001 | A | 13 | <5 | >120 | >1 | 0.2 | |
| 2002 | A | 48 | <5 | >120 | >1 | 0.2 | |
| 2004 | R | 336 | <5 | >120 | >1 | 0.50 | |
| 2005 | A | 23 | <5 | >120 | >1 | 0.08 | |
| Patient 1 | 2006-2018 | R | 276-405 | <5 | >120 | >1 | 0.50-0.55 |
| Post-splenectomy | |||||||
| Patient 2 | 1994-2006 | A | 7-41 | <5 | >120 | >1 | 0.06-0.3 |
| Pre-splenectomy | (9 episodes) | ||||||
| 2005-2006 | R | 198-290 | <5 | >120 | >1 | 0.7-0.9 | |
| Patient 2 | 2006 | R | 371 | <5 | >120 | 0.7 | |
| Post-splenectomy | 2007 | A | 88 | <5 | >120 | >1 | 0.4 |
| 2008-2018 | R | 186-339 | <5 | >120 | 0.7-1 |
NA, not available; NV, normal value; BU, Bethesda Units. *Acute episodes were always associated with laboratory data consistent with haemolytic anaemia. °Ranges are provided when multiple episodes were considered. Normal values were considered levels of ADAMTS13 activity and antigen measured in 50 control patients