COVID-19 as a Potential Trigger for Immune Thrombotic Thrombocytopenic Purpura and Reason for an Unusual Treatment: A Case Report
Schwaegermann, M.-K.; Hobohm, L.; Rausch, J.; Reuter, M.; Griemert, T.-F.; Sivanathan, V.; Falter, T.; Sprinzl, M.-F.; Lackner, K.-J.; Galle, P.-R.; Konstantinides, S.; Theobald, M.; von Auer, C. · Zeitschrift für Orthopädie und Unfallchirurgie · 2023 · Heft 03 · S. 215 bis 218
Bibliografische Angaben
Zusammenfassung
AbstractImmune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, i…