This handout is for thrombotic thrombocytopenic purpura (immune-mediated). Your care team identified this based on: thrombocytopenia + maha (schistocytes + ldh up + indirect bili up) — ash 2020; isth 2017.
Other reasons your team may use this plan: pentad: thrombocytopenia + maha + neuro + renal + fever (rare complete) — ash 2020; high plasmic score → adamts13 send-out + stat pex — bendapudi 2017; ash 2020.
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| plasma_exchange_TPE | 1.5 plasma volumes per session, exchanged with FFP or solvent-detergent plasma | apheresis | daily until plt >150 × 2 days | Rock NEJM 1991 — mortality 90%→10%; mainstay even before ADAMTS13 result |
Plan: Acute iTTP induction (PLEX + caplacizumab + steroid + rituximab — HERCULES NEJM 2019)
Call 911 or go to the nearest emergency room right away if you have:
Hem clinic for ADAMTS13 surveillance; vaccination; preventive rituximab if low ADAMTS13 trend; pregnancy risk counseling — ASH 2020; ISTH 2017
Guideline: ISTH 2020 TTP Guidelines — diagnosis (Zheng et al, J Thromb Haemost 2020) + treatment; 2025 ISTH focused update; Rock et al NEJM 1991 plasma exchange (Canadian Apheresis Study Group); HERCULES caplacizumab (Scully NEJM 2019); PLASMIC score (Bendapudi Lancet Haematol 2017)