Levy GG, Nichols WC, Lian EC, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura[J]. Nature, 2001, 413(6855): 488-494.
[2]
Schneppenheim R, Budde U. von Willebrand factor: the complex molecular genetics of a multidomain and multifunctional protein[J]. J Thromb Haemost, 2011, 9 (Suppl 1): 209-215.
[3]
Luken BM, Turenhout EA, Hulstein JJ, et al. The spacer domain of ADAMTS13 contains a major binding site for antibodies in patients with thrombotic thrombocytopenic purpura[J]. J Thromb Haemost, 2005, 93(2): 267-274.
[4]
Soejima K, Nakagaki T. Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies[J]. Semin Hematol, 2005, 42(1): 56-62.
[5]
Klaus C, Plaimauer B, Studt JD, et al. Epitope mapping of ADAMTS13 autoantibodies in acquired thrombotic thrombocytopenic purpura[J]. Blood, 2004, 103(12): 4514-4519.
[6]
Romani De Wit T, Fijnheer R, Brinkman HJ, et al. Endothelial cell activation in thrombotic thrombocytopenic purpura (TTP): a prospective analysis[J]. Br J Haematol, 2003, 123(3): 522-527.
[7]
Gadisseur A, Hermans C, Berneman Z, et al. Laboratory diagnosis and molecular classification of von Willebrand disease[J]. Acta Haematol, 2009, 121(2-3): 71-84.
[8]
Wagner DD, Fay PJ, Sporn LA, et al. Divergent fates of von Willebrand factor and its propolypeptide (von Willebrand antigen II) after secretion from endothelial cells[J]. Proc Natl Acad Sci,1987,84(7):1955-1959.
[9]
Vesely SK, George JN, Lammle B, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients[J]. Blood, 2003, 102(1): 60-68.
[10]
Nangaku M, Nishi H, Fujita T. Pathogenesis and prognosis of thrombotic microangiopathy[J]. Clin Exp Nephrol, 2007, 11(2): 107-114.
[11]
Veyradier A, Obert B, Houllier A, et al. Specific von Willebrandfactor-cleaving protease in thrombotic microangiopathies: a study of 111 cases[J]. Blood, 2001, 98(6): 1765-1772.
[12]
Zheng XL, Kaufman RM, Goodnough LT, et al. Effect of plasma exchangeon plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura[J]. Blood, 2004, 103(11): 4043-4049.
[13]
Kremer Hovinga JA, Studt JD, Lammle B. The von Willebrandfactor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP)[J]. Pathophysiol Haemost Thromb, 2003, 33(5-6): 417-421.
[14]
Kim JW, Kim I, Oh KH, et al. Therapeutic plasma exchange in patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the 10-year experience of a single center[J]. Hematology, 2011, 16(2): 73-79.
[15]
Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura[J]. Blood, 2008, 112(1): 11-18.
[16]
Burns ER, Zucker-Franklin D. Pathologic effects of plasma from patients with thrombotic thrombocytopenic purpura on platelets and cultured vascular endothelial cells[J]. Blood, 1982, 60(4): 1030-1037.
[17]
Lotta LA, Lombardi R, Mariani M, et al. Platelet reactive conformation and multimeric pattern of von Willebrand factor in acquired thrombotic thrombocytopenic purpura during acute disease and remission[J]. J Thromb Haemost, 2011, 9(9): 1744-1751.
van Mourik JA, Romani de Wit T. Von Willebrand factor pro-peptide in vascular disorders[J]. Thromb Haemost, 2001,86(1): 164-171.
[20]
Chauhan AK, Walsh MT, Zhu G, et al. The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis[J]. Blood, 2008, 111(7): 3452-3457.
Mori Y, Wada H, Gabazza EC, et al. Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity[J]. Transfusion, 2002, 42(5): 572-580.
[23]
Borchiellini A, Fijnvandraat K, ten Cate JW, et al. Quantitative analysis of von Willebrand factor propeptide release ？in vivo？: Effect of experimental endotoxemia and administration of 1-deamino-8-D-arginine vasopressin in humans[J]. Blood, 1996, 88(8): 2951-2958.
[24]
Bohm M, Betz C, Miesbach W, et al. The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine[J]. Br J Haematol, 2005, 129(5): 644-652.
