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Wolfgang Miesbach: Caplacizumab as Frontline Therapy for iTTP

Wolfgang Miesbach/LinkedIn

Dec 20, 2025, 12:07

Wolfgang Miesbach: Caplacizumab as Frontline Therapy for iTTP

Wolfgang Miesbach, Professor of Medicine at Frankfurt University Hospital, shared on LinkedIn:

“Caplacizumab as Frontline Therapy for iTTP: Is Plasmapheresis Still Needed? Priv.-Doz. Dr.med. Linus Völker presented at ASH compelling evidence that caplacizumab can work without plasma exchange in selected patients.

Systematic evidence: >100 plasma-free iTTP episodes

First case reports and small series: ~10 episodes
Kühne et al., Blood 2024 (Austria/Germany): 42 episodes with 90.5% clinical response without TPE and a median time to platelet normalization: 3 days (vs. 4 days with TPE, p=0.31), however, 4 patients required rescue TPE
Knöbl, ISTH 2025: 34 episodes
MAYARI Trial, ISTH 2025: 46 episodes and 93.5% remission without TPE (43/46 patients), 95.7% remission overall (44/46 patients)

This strategy is off-label and requires institutional capabilities:

Extensive iTTP treatment experience at specialized centers
24/7 ADAMTS13 activity measurement (turnaround in hours, not days)
Immediate caplacizumab access via emergency pharmacy
Plasma exchange instantly available (safety net, not routine)
No contraindications to caplacizumab therapy

Crucial to identify the right patient
There are clinical characteristics favoring a plasma-free approach

Immediate platelet recovery after the first caplacizumab dose
Clinically stable condition at presentation
Lower LDH levels at onset
Less severe organ involvement (lower ICU admission rates)

A fascinating glimpse into how frontline caplacizumab, in the right setting with the right patient, may allow truly plasma-free management for selected iTTP cases.”

Wolfgang Miesbach