Acquired thrombotic thrombocytopenic purpura
Studt, J.-D.; Voorberg, J.; Hovinga, J.-A.-K.; Schaller, M. · Zeitschrift für Orthopädie und Unfallchirurgie · 2013 · Heft 02 · S. 121 bis 130
Bibliografische Angaben
Zusammenfassung
SummaryThe von Willebrand factor (VWF)-cleaving metalloprotease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motifs-13) is the only known target of the dysregulated immune response in acquired TTP. Autoantibodies to ADAMTS13 either neutralize its activity or accelerate its clearance, thereby causing a severe deficiency of ADAMTS13 in plasma. As a consequence, size regulation of VWF is impaired and the persistence of ultra-large VWF (ULVWF) multimers facilitates micro vascular platelet aggregation causing microangiopathic haemolytic anaemia and ischaemic organ damage. Autoimmune TTP alt…
