First TTP guidelines released at ISTH 2020

A two-year development process has delivered the first ISTH Guidelines for Treatment of Thrombotic Thrombocytopenic Purpura (TTP).

Presented at the ISTH 2020 Virtual Congress and published in the Journal of Thrombosis and Haemostasis, the guidelines include 11 recommendations on the management of TTP although most were based on low certainty evidence.

Nevertheless strong recommendations were made for the addition of corticosteroids to therapeutic plasma exchange for the management of a first acute event and during a relapse.

Conditional recommendations, in the context of very low evidence, were made for the addition of rituximab to corticosteroids and plasma exchange in order to reduce the risk of relapse.

Co-chair of the international multidisciplinary guideline panel Professor Long Zheng from the University of Kansas, told the Congress that another conditional recommendation was for the use of caplacizumab for the management of a first acute event and during a relapse.

However caplacizumab does not correct the underlying plasma ADAMTS13 deficiency nor does it eliminate autoantibodies against ADAMTS13, the guidelines said.

“The evidence is really coming from a randomised double blind trial published in the NEJM, that demonstrated that caplacizumab reduced the time to normalisation of platelet count as the primary outcome,” Professor Zheng said.

Caplacizumab may also reduce exacerbations as well as improve some composite outcomes including TTP-related death, recurrence of TTP, or a major thromboembolic event, he said.

“For patients with iTTP who are in remission, but still have low ADAMTS13 activity with no clinical signs or symptoms, the Panel suggests the use of rituximab over non-use of rituximab for prophylaxis.”

The Panel recommended either plasma infusion or a watch and wait strategy for patients with congenital TTP who were in remission, and against the use of factor VIII concentrate infusions.

And there were two specific recommendations for the management of immune TTP and congenital TTP in pregnancy.

“For patients with iTTP who are pregnant and have decreased plasma ADAMTS13 activity but with no clinical signs or symptoms, the panel recommends prophylactic treatment over no prophylactic treatment,” Professor Zheng said.

Similarly for patients with cTTP who are pregnant, there was a strong recommendation for prophylactic treatment and a conditional recommendation for plasma infusion rather than factor VIII products.

Professor Zheng said further research was required on how to monitor and act on ADAMTS13 levels in remission and comparative studies between various treatment approaches.

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