Therapeutic plasma exchange in thrombotic thrombocytopenic purpura
Copyright © 2019 Elsevier Masson SAS. All rights reserved..
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure. The disease is accompanied with microangiopathic haemolytic anaemia, consumptive thrombocytopenia and lies on a severe deficiency in ADAMTS13, the von Willebrand factor-cleaving protease. In the acquired, immune-mediated form, this deficiency is due to the production of autoantibodies directed against the enzyme. Therapeutic plasma exchange has been used empirically for decades and still represents the cornerstone of TTP treatment. However, a better understanding of pathophysiological mechanisms underlying the disease has led these last years to the development of highly effective targeted therapies that might in the future restraint the use of therapeutic plasma exchange.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:48 |
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| Enthalten in: |
Presse medicale (Paris, France : 1983) - 48(2019), 11 Pt 2 vom: 15. Nov., Seite 319-327 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Picod, Adrien [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 11.12.2019 Date Revised 17.12.2019 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1016/j.lpm.2019.08.024 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM303623225 |
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| 520 | |a Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease related to the formation of microvascular thrombosis and subsequent organ failure. The disease is accompanied with microangiopathic haemolytic anaemia, consumptive thrombocytopenia and lies on a severe deficiency in ADAMTS13, the von Willebrand factor-cleaving protease. In the acquired, immune-mediated form, this deficiency is due to the production of autoantibodies directed against the enzyme. Therapeutic plasma exchange has been used empirically for decades and still represents the cornerstone of TTP treatment. However, a better understanding of pathophysiological mechanisms underlying the disease has led these last years to the development of highly effective targeted therapies that might in the future restraint the use of therapeutic plasma exchange | ||
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