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Region Hovedstaden - en del af Københavns Universitetshospital

Good practice statements (GPS) for the clinical care of patients with thrombotic thrombocytopenic purpura

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review


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  • X Long Zheng
  • Sara K Vesely
  • Spero R Cataland
  • Paul Coppo
  • Brian Geldziler
  • Alfonso Iorio
  • Masanori Matsumoto
  • Reem A Mustafa
  • Menaka Pai
  • Gail Rock
  • Lene Russell
  • Rawan Tarawneh
  • Julie Valdes
  • Flora Peyvandi
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BACKGROUND: Despite advances in treatment options for thrombotic thrombocytopenic purpura (TTP), there are still limited high quality data to inform clinicians regarding its management.

METHODS: In June 2018, the ISTH formed a multidisciplinary guideline panel to issue recommendations about treatment of TTP. The panel discussed 12 treatment questions related to both immune-mediated TTP (iTTP) and hereditary/congenital TTP (cTTP). The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including evidence-to-decision frameworks, to appraise evidence and formulate recommendations.

RESULTS: The panel agreed on eleven recommendations based on evidence ranging from very low to moderate certainty. For first episode and relapses of acute iTTP, the panel made a strong recommendation for the addition of corticosteroids to therapeutic plasma exchange (TPE), and a conditional recommendation for addition of rituximab and caplacizumab. For asymptomatic iTTP with low ADAMTS13, the panel made a conditional recommendation for rituximab outside of pregnancy, and for prophylactic TPE during pregnancy. For asymptomatic cTTP, the panel made a strong recommendation for prophylactic plasma infusion during pregnancy, but a conditional recommendation for plasma infusion or a wait and watch approach outside of pregnancy.

CONCLUSIONS: The panel's recommendations are based on all the available evidence for the treatment effects of various approaches including suppressing inflammation, blocking platelet clumping, replacing the missing and/or inhibited ADAMTS13, and suppressing ADAMTS13 antibody production. There was insufficient evidence for further comparison of different treatment approaches, for which future high-quality studies in iTTP (e.g., rituximab, corticosteroids, recombinant ADAMTS13, and caplacizumab) and in cTTP (eg, recombinant ADAMTS13) are needed.

TidsskriftJournal of thrombosis and haemostasis : JTH
Udgave nummer10
Sider (fra-til)2503-2512
Antal sider10
StatusUdgivet - okt. 2020

Bibliografisk note

© 2020 International Society on Thrombosis and Haemostasis.

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