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Thrombotic Thrombocytopenic Purpura (TTP) — Treatment
First-line
Second-line
SECOND-LINE
Acquired TTP
ADAMTS13 < 10%
(immune)
Acute episode
Immunomodulation
Relapse prevention
Emergent
Plasma exchange (PLEX)
daily until recovery
Immunosuppress
Corticosteroids
+ PLEX
Anti-VWF
Caplacizumab
HERCULES ↗
Anti-CD20
Rituximab
frontline adjunct / relapse prevention ↗
Preemptive
Rituximab for falling ADAMTS13
in remission ↗