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References

References

1. Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. BLOOD 2008;112:11-8

2. Sarode R, Bandarenko N, Brecher ME, Kiss JE, Marques MB, Szczepiorkowski ZM, Winters JL. Thrombotic thrombocytopenic purpura : 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J Clin  Apher 2014;29:148-67 

3. Moake JL. Mechanisms of Disease: Thrombotic microangiopathies. N Engl J Med 2002;347:589-600 
 
4. Terell DR, Williams LA, Vesely SK, Lämmle B, Kremer Hovinga JA, George JN. T he incidence of thrombotic thrombocytopenic purpura – hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiencyJ Thromb Haemost 2005;3:1432-6 
 
5. Reese JA, Muthurajah DS, Kremer Hovinga JA, Vesely SK, Terrell DR, George JN. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired ADAMTS13 deficiency: comparison of incidence, demographic and clinical features. Pediatr Blood Cancer 2013;60:1-7 
 
6. Crawley JTB, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program 2013;2013:292-9 
  
7. Schneppheim R, Kremer Hovinga JA, Becker T, Budde U, Karpman D, Brockhaus W, Harchovinová I, Korczowski B, Oyen F, Rittich S, von Rosen J, Tjønnfjord GE, Pimanda JE, Wienker TF, Lämmle B. A common origin of the 4143insA ADAMTS13 mutationThromb Haemost 2006;96: 3-6  

8.  Camilleri RS, Cohen H, Mackie IJ, Scully M, Starke RD, Crawley JTB, Lane DA, Machin SJ. Prevalence of the ADAMTS-13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpuraJ Thromb Haemost 2008;6:331-8 
  
9. Plaimauer B, Fuhrmann J, Mohr G, Wernhart W, Bruno K, Ferrari S, Konetschny C, Antoine G, Rieger M, Scheiflinger F. Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation. BLOOD 2006;107:118-24 
 
10. Lancellotti S, Bassao M, De Cristofaro R. Proteolytic Processing of Von Willebrand Factor by ADAMTS13 and Leukocyte Proteases. Mediterr J Hematol Infect Dis 2013;5:e2013058 
  
11. Galbusera M, Noris M, Remuzzi G. Thrombotoic thrombocytopenic Purpura – Then and Now. Semin Thromb Hemost 2006;32:81-9  

12. Zhou Z, Xeh H, Jing H, Wang C, Tao Z, Choi H, Aboulfatova K, Li R, Dong JF. Cystein residues in CUB-1 domain are critical for ADAMTS13 secretion and stabilityThromb Haemost 2011;105:21-30

13. Lotta LA, Wu HM, Mackie IJ, Noris M, Veyradier A, Scully MA, Remuzzi G, Coppo P, Liesner R, Donadelli R, Loirat C, Gibbs RA, Horne A, Yang S, Garagiola I, Musallam KM, Peyvandi F. Residual plasmatic activity of ADAMTS13 is correlated with phenotype severity in congenital thrombotic thrombocytopenic purpura. BLOOD 2012;120:440-8

14. Lotta LA , Garagiola I, Palla R, Cairo A, Peyvandi F. ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura.  Hum Mutat 2010;31:11-9

15. Manea M, Kristoffersson A, Schneppenheim R, Saleem MA, Mathieson PW, Mörgelin M, Björk P, Holmberg L, Karpman D. Podocytes express ADAMTS13 in normal renal cortex and in patients with thrombotic thrombocytopenic purpura. Br J Haematol 2007;138:651-62 
  
16. Tauchi R, Imagama S, Ohgomori T, Natori T, Shinjo R, Ishiguro N, Kadomatsu K. ADAMTS-13 is produced by glial cells and upregulated after spinal cord injury. Neurosci Lett 2012;517:1-6 
 
17. Furlan M, Robles R, Lämmle B. Partial Purification and Characterization of a Protease From Human Plasma Cleaving von Willebrand Factor to Fragments Produced by In Vivo ProteolysisBLOOD 1996;87:4223-34 
 
18. Kremer Hovinga JA, B. Lämmle B. Role of ADAMTS13 in the pathogenesis, diagnosis and treatment of thrombotic thrombocytopenic purpuraHematology Am Soc Hematol Educ Program 2012;2012:610-6 
 
19. Furlan M, Robles R, Morselli B, Sandoz P, Lämmle B. Recovery and Half-Life of von Willebrand Factor-Cleaving Protease after Plasma Therapy in Patients with Thrombotic Thrombocytopenic Purpura. Thromb Haemost 1999;81:8-13 
 
20. Jiang Y, Mclntosh JJ, Reese JA, Deford CC, Kremer Hovinga JA, Lämmle B, Terrell DR, Vesely SK, KnudtsonEJ, George JN. Pregnancy outcomes following recovery form acquired thrombotic thrombocytopenic purpura. BLOOD 2014;123:1674-80
 
21. George JN, Chen Q, Deford CC, Al-Nouri Z. Ten Patient Stories Illustrating the Extraordinarily Diverse Clinical Features of Patients With Thrombotic Thrombocyytopenic Purpura and Severe ADAMTS13 DeficiencyJ Clin Apher 2012;27:302-11 
 
22. Yamashita E, Okada H, Yorioka H, Fujita S, Nishi K, KomiyamaY, Kanzaki H. Successful management of pregnancy-associated thrombotic thrombocytopenic purpura by monitoring ADAMTS13 activityJ Obstet Gynaecol Res 2012;38:567-9 
 
23. Uemura M, Fujimura Y, Ko S, Matsumoto M, Nakajima Y, Fukui. Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis. Int J Hepatol 2011;2011:759047 
 
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26. Rock GA, Shumak KH, Buskard NA, Blanchette VS, Kelton JG, Nair RC, Spasoff RA, and the Canadian Apheresis Study Group. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpuraN Engl J Med 1991;325:393-7 

27.  Knöbl P. Inherited and Acquired Thrombotic Thrombocytopenic Purpura (TTP) in Adults. Semin Thromb Hemost 2014;40:493–502 
 
28. Schiviz A, Wuersch K, Piskernik C, Dietrich B, Hoellriegl W, Rottensteiner H, Scheiflinger F, Schwarz HP, Muchitsch E. A new mouse model mimicking thrombotic thrombocytopenic purpura: correction of symptoms by recombinant human ADAMTS13. BLOOD 2012;119:6128-35 
 
29. Holz JB. The TITAN trial – Assessing the efficacy and safety of an anti-von Willebrand factor Nanobody in patients with acquired thormbotic thrombocytopenic purpura. Transfus Apher Sci 2012;46:343-6 
 
30. Mansouri Taleghani M, von Krogh AS, Fujimura Y, George JN, Hrachovinova I, Knöbl PN, Quist-Paulsen P, Schneppenheim R, Lämmle B, Kremer Hovinga JA. Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry. Hamostaseologie 2013;33:138-43 
 
31. Upshaw JD. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia.  N Engl J Med 1978;298:1350-2 
 
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33. Tsai HM. Physiologic Cleavage of von Willebrand Factor by a Plasma Protease Is Dependent on Its Conformation and Requires Calcium Ion. BLOOD 1996;87:4235-44 
 
34. Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lämmle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. BLOOD 1997;89:3097-103
  
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