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Therapie der thrombotisch-thrombozytopenischen Purpura (TTP) bei kritisch kranken Patienten

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Book cover 27. Hämophilie-Symposion Hamburg 1996

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Zusammenfassung

Die thrombotisch-thrombozytopenische Purpura (TTP) wurde erstmals 1924 von Moschcowitz beschrieben [1]. Es ist eine seltene Erkrankung, die durch thrombozytäre Mikrothromben charakterisiert ist, welche zu Mikrozirkulationsstörungen und zur ischälnischen Organdysfunktion führen. Die am häufigsten betroffenen Organe sind Gehirn, Nieren, Herz, Lunge und Leber, es kann aber auch jedes andere Organ befallen sein. Die klassischen Leitsymptome sind Thrombopenie, hämolytische Anämie, Fieber, neurologische Ausfälle und Nierenversagen. Das hämolytisch-urämische Syndrom (HUS) [2] ist eine andere Ausprägung derselben Krankheitsgruppe (thrombotische Mikroangiopathien) mit vorwiegender Beteiligung der Nieren. Die Pathogenese dieser Krankheitsgruppe ist unklar. Allgemein wird jedoch angenommen, daß eine systemische Störung der Endothelzellfunktion [3] zu einer Beeinflussung der Synthese oder der intrazellulären Verarbeitung des v.-Willebrand-Faktors führt, was eine Ausschwemmung von ungewöhnlich großen v.-Willebrand-Faktor-Multimeren zur Folge hat, die verstärkt an die Plättchenrezeptoren GP Ib und GP IIb/IIIa binden und eine systemische Thrombozytenaggregation hervorrufen [4, 5]. Auslösende oder prädisponierende Faktoren sind Schwangerschaft [6], Infektionen (E. Coli, Shigellen, HIV oder andere Viren) [7], Medikamente (Penicillin, Mitomycin C, Cyclosporin A), Malignome, Organtransplantationen (Niere, Leber, Knochenmark) und Systemerkrankungen (Lupus erythematodes), aber in den meisten Fällen kann keine eindeutige Ursache identifiziert werden. Die Differentialdiagnose umfaßt die disseminierte intravasale Gerinnung, Eklampsie, HELLP-Syndrom, maligne Hypertension und schwere Vaskulitis [8–10].

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Authors
  1. P. Knöbl
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  2. C. Rintelen
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  3. G. Kornek
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  4. C. Wiltschke
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  5. S. Globits
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  6. P. Kahls
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  7. I. Schwarzinger
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  8. P. Kyrle
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  9. M. Frass
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Editors and Affiliations

  1. Zentr. d. Inn. Med., Med. Klinik I, Universitätsklinikum, Theodor-Stern-Kai 7, D-60590, Frankfurt am Main, Deutschland

    Inge Scharrer

  2. Hämostaseologische Abteilung, Med. Univ.-Klinik Innenstadt, Ziemssenstraße 1a, D-80336, München, Deutschland

    Wolfgang Schramm

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© 1998 Springer-Verlag Berlin Heidelberg

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Knöbl, P. et al. (1998). Therapie der thrombotisch-thrombozytopenischen Purpura (TTP) bei kritisch kranken Patienten. In: Scharrer, I., Schramm, W. (eds) 27. Hämophilie-Symposion Hamburg 1996. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-80403-8_16

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  • DOI: https://doi.org/10.1007/978-3-642-80403-8_16

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-62915-3

  • Online ISBN: 978-3-642-80403-8

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