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

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  1. Reply to: Clinical impact of high platelet count and high hematocrit, by Marc Sorigue

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  2. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura

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  3. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura

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  4. Plasma levels of mannose-binding lectin and future risk of venous thromboembolism

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  1. Thrombocytopenia in intensive care unit patients: A scoping review

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  2. Low-dose hydrocortisone in patients with COVID-19 and severe hypoxia (COVID STEROID) trial-Protocol and statistical analysis plan

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  3. Correction to: Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura

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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.

Original languageEnglish
JournalJournal of thrombosis and haemostasis : JTH
Volume18
Issue number10
Pages (from-to)2503-2512
Number of pages10
ISSN1538-7933
DOIs
Publication statusPublished - 2020

ID: 61234111