The International Society on Thrombosis and Haemostasis (ISTH) recently released updated guidelines regarding the treatment of thrombotic thrombocytopenic purpura. The 2025 update also follows the previous, 2020 guidelines and follows their guidelines on the diagnosis of thrombotic thrombocytopenia.

The updated treatment guidelines focus, specifically, on managing congenital thrombotic thrombocytopenic purpura (cTTP). 

Ultimately, the panel that convened to review the latest research decided that no changes for the treatment of immune thrombotic thrombocytopenic purpura (iTTP) were warranted. However, cTTP management received some notable updates. We outlined those key elements below, for your convenience.

Key Elements of the 2025 Update:
  • Updated Recommendation 7 (changed from neutral to in favor of plasma infusion): For patients with cTTP who are in remission, the panel suggests prophylaxis with plasma infusion over a watch-and-wait strategy. (A conditional recommendation in the context of very low certainty evidence).
  • New Recommendation 8B: For patients with cTTP who are in remission, the guideline panel recommends recombinantADAMTS-13over plasma infusion to prevent acute episodes.
  • Updated Statement 19 (updated entirely): Prophylactic dosing of anticoagulants, most likely low molecular weight heparin, could be considered for patients with iTTP with recovered platelet counts >50 × 109/L, and for those with an increased risk of venous thrombosis (eg, history of recurrent VTE, cancer, and recent surgery). For the optimal management of patients with iTTP and major thrombotic events (eg, stroke, acute myocardial infarction), a multidisciplinary team including hematologists, neurologists, and cardiologists should be consulted.

The following recommendations received minor updates, but the direction/strength of the recommendation remained unchanged from the 2020 guidelines.

  • (Minor Updated) Recommendation 1: For patients with iTTP experiencing a first acute event, the Panel recommends the addition of corticosteroids to TPE over TPE alone. (A strong recommendation in the context of very low certainty evidence).
  • (Minor Updated) Recommendation 2: For patients with iTTP experiencing their first acute event, the panel suggests the addition of rituximab to corticosteroids and TPE over corticosteroids and TPE alone. (A conditional recommendation in the context of very low certainty evidence).
  • (Minor Updated) Recommendation 5: For patients with iTTP experiencing an acute event (first event or relapse), the panel suggests using caplacizumab over not using caplacizumab. (A conditional recommendation in the context of moderate certainty evidence).

Click here to view the full text of the article for a complete look at the guidelines.

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