Good practice statements (GPS) for the clinical care of patients with thrombotic thrombocytopenic purpura
| Autor: | Brian Geldziler, X. Long Zheng, Paul Coppo, Sara K. Vesely, Alfonso Iorio, Flora Peyvandi, Lene Russell, Spero R. Cataland, Menaka Pai, Julie Valdes, Gail Rock, Reem A. Mustafa, Rawan Tarawneh, Masanori Matsumoto |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Thrombotic thrombocytopenic purpura ADAMTS13 Protein 030204 cardiovascular system & hematology Asymptomatic Article 03 medical and health sciences 0302 clinical medicine Adrenal Cortex Hormones hemic and lymphatic diseases medicine Humans Intensive care medicine First episode Pregnancy Plasma Exchange Purpura Thrombotic Thrombocytopenic business.industry Hematology Guideline Single-Domain Antibodies medicine.disease ADAMTS13 Rituximab medicine.symptom Caplacizumab business medicine.drug |
| Zdroj: | J Thromb Haemost |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|