Acquired thrombotic thrombocytopenic purpura

Journal: Hämostaseologie
ISSN: 0720-9355

Thrombotic microangiopathies

Issue: Issues of 2013 (Vol. 33): Issue 2 2013 (77-188)
Pages: 121-130

Acquired thrombotic thrombocytopenic purpura

Development of an autoimmune response

M. Schaller (1), J.-D. Studt (2), J. Voorberg (3), J. A. Kremer Hovinga (1)

(1) Department of Haematology and Central Haematology Laboratory, Haemostasis Research Laboratory, Inselspital, Bern University Hospital and University of Bern, Switzerland; (2) Division of Haematology, University Hospital Zürich, Switzerland; (3) Department of Plasma Proteins, Sanquin-AMC Landsteiner Laboratory, Amsterdam, The Netherlands


autoimmunity, ADAMTS13, TTP, autoantibodies, Thromobotic thrombocytopenic purpura


The von Willebrand factor (VWF)-cleaving metalloprotease, ADAMTS13 (adisintegrin 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. 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.

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