Table 5.
Various therapies and recorded outcome in 32 cases with different ADAMTS 13 activity
| Various therapies | ADAMTS 13 activity | ADAMTS 13 activity | ADAMTS 13 activity | ADAMTS 13 activity |
|---|---|---|---|---|
| (<5 % of normal) | (10–25 %) | (25–50 %) | (>50 %) | |
| (N = 13) | (N = 2) | (N = 12) | (N = 5) | |
| PE alone | NR | NR | 1 died | 1 Rfa |
| ST and (or) cytotoxics | NR | 1 Rm | 1 Rm | 1 Rfa |
| PE + ST pulse therapy | 7 Rm, 2 Rfa | 1 Rm | 1 Rm | NR |
| 2 died | ||||
| PE + cytotoxics | 1 Rm | NR | 1 Rm | NR |
| PE + ST pulse therapy + cytotoxics | 2 Rm | NR | 6 Rm | 1 Rm |
| Rituximab + PE with or without ST (or cytotoxics) | 1 Rm | NR | 2 Rm | 3 Rm |
| rTM | NR | NR | NR | 1 Rm |
PE plasma exchange and (or) hematodialysis and (or) plasma infusion, ST steroids (glucocorticoids), mostly with pulse methylprednisolone therapy, cytotoxics including cyclophosphamide, mycophenolate mofetil, vincristine, cyclosporine A, and methotrexate, rTM recombinant human soluble thrombomodulin, Rf refractory, Rm remission, NR not reported
aThe patient was refractory to one therapy but achieved remission by additional therapy such as cytotoxics or rituximab