European Renal Best Practice endorsement of guidelines for diagnosis and therapy of thrombotic thrombocytopaenic purpura published by the International Society on Thrombosis and Haemostasis

Kathrin Eller; Paul Knoebl; Sevcan A Bakkaloglu; Jan J Menne; Paul T Brinkkoetter; Leonie Grandt; Ursula Thiem; Paul Coppo; Marie Scully; Maria C Haller

doi:10.1093/ndt/gfac034

European Renal Best Practice endorsement of guidelines for diagnosis and therapy of thrombotic thrombocytopaenic purpura published by the International Society on Thrombosis and Haemostasis

Nephrol Dial Transplant. 2022 Jun 23;37(7):1229-1234. doi: 10.1093/ndt/gfac034.

Authors

Affiliations

¹ Division of Nephrology, Medical University of Graz, Graz, Austria.
² Division of Haematology and Haemostasis, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
³ Department of Paediatric Nephrology, Gazi University, Faculty of Medicine, Ankara, Turkey.
⁴ KRH Klinikum Mitte - Location Siloah, Hannover, Germany.
⁵ Department II of Internal Medicine and Centre for Molecular Medicine Cologne (CMMC), Faculty of Medicine and University Hospital of Cologne, University of Cologne, Cologne, Germany.
⁶ Department of Medicine III - Nephrology, Ordensklinikum Linz Elisabethinen, Linz, Austria.
⁷ Department of Haematology, Reference Centre for Thrombotic Microangiopathies, Saint-Antoine University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
⁸ Department of Haematology, University College London Hospitals, London, UK.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that is caused by severe ADAMTS-13 deficiency. Immune-mediated TTP develops due to autoantibodies against ADAMTS-13, whereas congenital TTP is caused by mutations in the ADAMTS13 gene. Diagnostic possibilities and treatment options in TTP have emerged in recent years, which prompted the International Society on Thrombosis and Haemostasis (ISTH) to publish clinical practice guidelines for the diagnosis and treatment of TTP in 2020. In this article, the European Renal Best Practice Working Group endorsed the ISTH guidelines and emphasizes a number of considerations, including the importance of rapid ADAMTS-13 activity testing, the use of rituximab and anti-von Willebrand factor therapies such as caplacizumab, that enhance the clinical applicability of the guidelines in Europe.

Keywords: AKI; guidelines; plasma exchange; systematic review; thrombotic microangiopathy.

MeSH terms

ADAMTS13 Protein
Hemostasis
Humans
Purpura, Thrombotic Thrombocytopenic* / diagnosis
Purpura, Thrombotic Thrombocytopenic* / etiology
Purpura, Thrombotic Thrombocytopenic* / therapy
Thrombosis* / diagnosis
Thrombosis* / etiology
Thrombosis* / therapy
von Willebrand Factor

Substances

von Willebrand Factor
ADAMTS13 Protein