Response to TPE and mortality in 44 patients with TMA due to severe ADAMTS13 deficiency according to the PLASMIC-S
Variables | PLASMIC-S risk assessment | ||
---|---|---|---|
Low-to-intermediate risk (N=8) | High risk (N=36) | ||
ITR, N (%) | 4 (50.0) | 31 (86.1) | 0.042 |
N of TPE for ITR, median (range) | 8.0 (7.0–9.0) | 5.0 (1.0–8.0) | 0.001 |
Death, N (%) | 4 (50.0) | 6 (16.7) | 0.064 |
*
Abbreviations: TPE, therapeutic plasma exchange; TMA, thrombotic microangiopathy; ADAMTS13, a disintegrin and metalloprotease with thrombospondin type 1 motif, member 13; PLASMIC-S, PLASMIC score; ITR, initial treatment response.
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