Acquired thrombotic thrombocytopenic purpura Development of an autoimmune response

作者：Schaller M^*; Studt J D; Voorberg J; Hovinga J A Kremer

来源：Hamostaseologie, 2013, 33(2): 121-+.

DOI：10.5482/HAMO-12-12-0023

• Thrombotic thrombocytopenic purpura
• TTP
• ADAMTS13
• autoimmunity
• autoantibodies

The 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 microvascular platelet aggregation causing microangiopathic haemolytic anaemia and ischaemic organ damage. Autoimmune TTP although a rare disease with an annual incidence of 1.72 cases has a mortality rate of 20% even with adequate therapy. %26lt;br%26gt;We describe the mechanisms involved in ADAMTS13 autoimmunity with a focus on the role of B- and T-cells in the pathogenesis of this disorder. We discuss the potential translation of recent experimental findings into future therapeutic concepts for the treatment of acquired TTP.

• 出版日期2013-6