Caplacizumab and/or Rituximab for TTP

By William Aird

The following poll was posted on Twitter Jan 25, 2024:

Interestingly, the leading response was neither!

Let’s look at what the guidelines say:

2020 International Society on Thrombosis and Haemostasis (ISTH):

The ISTH recommends using both rituximab and caplacizumab for acquired TTP, first acute event (conditional recommendation).

2023 British Society of Haematology (BSH):

The BSH recommends initiating therapy with monoclonal anti-CD20 therapy within 3 days of acute acquired TTP (1B), and caplacizumab upon confirmation of acute iTTP (1A).

UpToDate (expert opinion):1

UTD recommends initiating rituximab once acquired TTP is confirmed, and caplacizumab for those with acquired TTP and severe features (though they acknowledge a broad range of preferences for using caplacizumab).

Primary studies: