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Thrombotische Mikroangiopathie und Niere

Thrombotic microangiopathy and the kidneys

  • Leitthema
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Der Nephrologe Aims and scope

Zusammenfassung

Die thrombotische Mikroangiopathie (TMA) ist eine schwere Erkrankung, die sich in einer Vielzahl von klinischen Manifestationen darstellen kann. Sie ist charakterisiert durch eine Thrombozytopenie, eine hämolytische Anämie, eine Mikroangiopathie und, damit verbunden, Organschaden durch Ischämie und Gewebsuntergang. In den letzten Jahren sind die molekularen Mechanismen der unterschiedlichen Formen der TMA besser verstanden worden und haben zu einer neuen Klassifikation der Erkrankung geführt. Man unterscheidet die thrombotische thrombozytopenische Purpura (TTP), das atypische hämolytisch-urämische Syndrom (aHUS) und die infektassoziierte TMA (STEC-HUS). Daneben kann die TMA durch unterschiedliche exogene Faktoren wie Medikamente ausgelöst werden. Häufig ist bei der TMA die Niere betroffen, aber es können auch neurologische, pulmonale und kardiovaskuläre Störungen auftreten. Die TMA ist mit einer hohen Morbidität und Mortalität verknüpft. Eine frühe systematische Diagnostik und die rasche Einleitung einer supportiven Therapie können, zusammen mit neuen Behandlungsmethoden, die Prognose der Erkrankung deutlich verbessern. Im Folgenden werden die Klassifikation der Erkrankung dargestellt, die Pathologie erörtert sowie die Pathogenese und die verschiedenen Ansätze der Therapie dargestellt.

Abstract

Thrombotic microangiopathy (TMA) is a severe, life-threatening disease which can be manifested in a diverse range of clinical symptoms. The TMA is characterized by thrombocytopenia, hemolytic anemia, microangiopathy and associated organ damage due to ischemia and tissue destruction. Only recently a better understanding of the molecular mechanisms of the different forms of TMA has led to a new classification of the disease. Thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS) and infection-associated TMA (STEC-HUS) are the major clinical entities of the disease. In addition, TMA can be triggered by various exogenic factors, such as medications. The kidneys are often affected in TMA but neurological, pulmonary and cardiovascular disorders can also occur. The TMA is associated with a significant morbidity and mortality. Early systematic diagnostics and rapid initiation of supportive treatment together with novel treatment methods can greatly improve the prognosis of the disease. This review describes the classification of the different forms of TMA, the underlying pathophysiology and pathogenesis as well as the various treatment approaches.

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  1. Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    H. Haller

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H. Haller hat Vortragshonorare von folgenden Firmen erhalten: Alexion, Astra-Zeneca, Bayer, Böhringer, Fresenius, Vifor-Pharma.

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H. Haller, Hannover

M. Haubitz, Fulda

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Haller, H. Thrombotische Mikroangiopathie und Niere. Nephrologe 14, 100–107 (2019). https://doi.org/10.1007/s11560-019-0320-4

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