Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency

Summary

We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission.

Pathomechanisms of thrombotic thrombocytopenic purpura, especially the role of ADAMTS-13, are discussed and therapeutic measures outlined.

Zusammenfassung

Wir berichten über eine Frau (Alter: 64 Jahre) mit akuter thrombotischer Mikroangiopathie bei schwerem akquiriertem Mangel an von-Willebrand-Faktor-spaltender Protease (ADAMTS-13). Sie wurde erfolgreich mit Plasmapheresen und Glukokortikosteroiden behandelt. Sieben Monate später kam es zu einem Rezidiv, eine Splenektomie wurde vorgenommen und führte zu anhaltender Remission.

Aktuelle Kenntnisse zur Pathophysiologie der thrombotisch-thrombozytopenischen Purpura werden diskutiert, insbesondere die Rolle von ADAMTS-13. Abschließend werden therapeutische Maßnahmen vorgestellt.

Keywords

ADAMTS-13 - microangiopathy - thrombotic thrombocytopenic purpura - von Willebrand factor-cleaving protease

Schlüsselwörter

Mikroangiopathie - thrombotisch-thrombozytopenische Purpura - von-Willebrand-Faktor-spaltende Protease - ADAMTS-13

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