Julia Arndt, M.Sc.
Phone: +49 (0)711 - 2 29 87 33
Fax: +49 (0)711 - 2 29 87 50
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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.
Elena Bresin 1; Sara Gastoldi 1; Erica Daina 1; Daniela Belotti 2; Enrico Pogliani 2; Paolo Perseghin 3; Potito R. Scalzulli 4; Rossella Paolini 5; Raimondo Marcenò 6; Giuseppe Remuzzi 1,7; Miriam Galbusera 1
Thromb Haemost 2009 101 2: 233-238
Brenda M. Luken 1, Ellen A. M. Turenhout 1, Paul H. P. Kaijen 1, Mascha J. Greuter 1, Wouter Pos 1, Jan A. van Mourik 1,3, Rob Fijnheer 2, Jan Voorberg 1
Thromb Haemost 2006 96 3: 295-301
Reinhard Schneppenheim 1, Johanna A. Kremer Hovinga2 , Tim Becker3 , Ulrich Budde4 , Diana Karpman 5, Wolfgang Brockhaus6 , Ingrid Hrachovinová 7, Bartosz Korczowski8 , Florian Oyen 1 , Šimon Rittich7 , Johannes von Rosen 9, Geir E. Tjønnfjord 10, J
Thromb Haemost 2006 96 1: 3-6