Der Nephrologe

, Volume 3, Issue 6, pp 452–463 | Cite as

Thrombotische Mikroangiopathien

Leitthema
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Zusammenfassung

Thrombotische Mikroangiopathien (TMA) stellen eine heterogene Gruppe von Krankheitsbildern dar, gemeinsam ist ihnen ein akutes Syndrom aus mikroangiopathischer hämolytischer Anämie, Thrombozytopenie und Organmanifestationen. Während das diarrhöinduzierte hämolytisch-urämische Syndrom unverändert die häufigste TMA-Form darstellt, haben neue Einsichten zu nicht-Diarrhoe-induzierten (sog. D–induzierten) TMA-Formen in den letzten Jahren zu einer auf pathogenetischen Aspekten basierenden Einteilung geführt. Insbesondere weisen neue Erkenntnisse auf die Rolle einer reduzierten Aktivität der von-Willebrand-Faktor spaltenden Protease ADAMTS 13 in der Pathogenese der thrombotischen thrombozytopenen Purpura hin und auf die Rolle von defekten komplementregulatorischen Proteinen in der Pathogenese von D–hämolytisch-urämischen Syndromen. Erstmalig kann daher eine rational begründete Therapie eingesetzt werden (z. B. Immunsuppression bei Auto-Antikörpern gegen ADAMTS 13, Faktor-H-Substitution durch Plasmatherapie bei Mutationen im Faktor H). Dennoch ist zurzeit eine pathogenetisch basierte Einteilung der meisten übrigen TMA-Formen (z. B. im Gefolge von immunologischen Systemkrankheiten, Malignomen, Medikamenten oder nach Transplantationen) nach wie vor nicht möglich, dementsprechend ist die Therapie überwiegend empirisch.

Schlüsselwörter

Hämolytisch urämisches Syndrom Thrombotische thrombozytopene Purpura Nierenversagen Diarrhö Komplement 

Thrombotic microangiopathies

Abstract

Thrombotic microangiopathies (TMA) are a heterogenous group of diseases characterized by an acute syndrome of microangiopathic hemolytic anemia, thrombocytopenia, and organ manifestations. While the diarrhea-induced hemolytic uremic syndrome continues to represent the most common type of TMA, new insights in the case of non-diarrhea (D)-induced TMA types have recently led to a new classification based on pathogenetic mechanisms. These new insights point particularly to the role of reduced activity of the von Willebrand factor cleaving protease ADAMTS 13 in the pathogenesis of thrombotic thrombocytopenic purpura and also to the role of defects in complement-regulatory proteins in the pathogenesis of D-hemolytic uremic syndromes. For the first time it is possible to initiate a pathophysiologically based therapy in these cases (e.g. immunosuppression in cases of autoantibodies against ADAMTS 13, factor H substitution by plasma therapy in cases of mutated factor H). Nevertheless at present such pathogenesis-based classification is not possible in most other TMA types (e.g. in the course of immunologic diseases, malignancies, administered drugs, or following transplantations) and thus therapy remains largely empiric in such cases.

Keywords

Hemolytic uremic syndrome Thrombotic thrombocytopenic purpura Renal failure Diarrhea Complement 

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Abteilung für Nephrologie und Klinische ImmunologieUniversitätsklinikum der RWTH AachenAachenDeutschland
  2. 2.Institut für PathologieUniversitätsspital BaselBaselSchweiz

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