Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

Presse Medicale

Volumn 41, Issue 3 PART 2, 2012, Pages

  Coppo, Paul ^a   Veyradier, Agnès ^b,c  

^a HÔPITAL SAINT ANTOINE   (France)

^b HÔPITAL ANTOINE BÉCLÈRE   (France)

^c UNIVERSITÉ PARIS SACLAY   (France)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLSALICYLIC ACID; ANTITHROMBOCYTIC AGENT; AZATHIOPRINE; CLOPIDOGREL; CYCLOPHOSPHAMIDE; CYCLOSPORIN A; DIPYRIDAMOLE; IMMUNOGLOBULIN; IMMUNOMODULATING AGENT; METHYLPREDNISOLONE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PREDNISOLONE; RITUXIMAB; STEROID; TICLOPIDINE; VINCRISTINE; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ADJUVANT THERAPY; AGE DISTRIBUTION; ANEMIA; ANTICOAGULANT THERAPY; CLINICAL FEATURE; DIFFERENTIAL DIAGNOSIS; DISEASE ASSOCIATION; DISEASE DURATION; DISEASE EXACERBATION; DISEASE SEVERITY; DOSE RESPONSE; DRUG DOSE REDUCTION; DRUG EFFICACY; DRUG MECHANISM; DRUG MEGADOSE; DRUG SAFETY; DRUG WITHDRAWAL; ENZYME DEFICIENCY; ERYTHROCYTE TRANSFUSION; GENE MUTATION; GENETIC ASSOCIATION; HELLP SYNDROME; HEMOLYTIC ANEMIA; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; KIDNEY FAILURE; MALIGNANT NEOPLASTIC DISEASE; ONSET AGE; PLASMA TRANSFUSION; PLASMAPHERESIS; PREGNANCY DISORDER; RECURRENT DISEASE; RELAPSE; REMISSION; RISK REDUCTION; SALVAGE THERAPY; SHORT SURVEY; SPLENECTOMY; STEROID THERAPY; SURVIVAL RATE; THROMBOCYTOPENIA; THROMBOTIC THROMBOCYTOPENIC PURPURA; TREATMENT RESPONSE; UPSHAW SCHULMAN SYNDROME;

EID: 84857440257     PISSN: 07554982     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.lpm.2011.10.024     Document Type: Short Survey

References (90)

1. Furlan M., Robles R., Galbusera M., Remuzzi G., Kyrle P.A., Brenner B., et al. von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 1998, 339(22):1578-1584.
2. Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998, 339(22):1585-1594.
3. Feys H.B., Roodt J., Vandeputte N., Pareyn I., Lamprecht S., van Rensburg W.J., et al. Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon (Papio ursinus). Blood 2011, 116(12):2005-2010.
4. Coppo P., Busson M., Veyradier A., Wynckel A., Poullin P., Azoulay E., et al. HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians. J Thromb Haemost 2010, 8(4):856-859.
5. Levy G.G., Nichols W.C., Lian E.C., Foroud T., McClintick J.N., McGee B.M., et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001, 413(6855):488-494.
6. Motto D.G., Chauhan A.K., Zhu G., Homeister J., Lamb C.B., Desch K.C., et al. Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice. J Clin Invest 2005, 115(10):2752-2761.
7. Moake J.L., Rudy C.K., Troll J.H., Weinstein M.J., Colannino N.M., Azocar J., et al. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982, 307(23):1432-1435.
8. Moake J.L., Turner N.A., Stathopoulos N.A., Nolasco L.H., Hellums J.D. Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986, 78(6):1456-1461.
9. Ridolfi R.L., Bell W.R. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature. Medicine (Baltimore) 1981, 60(6):413-428.
10. Coppo P., Schwarzinger M., Buffet M., Wynckel A., Clabault K., Presne C., et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One 2010, 5(4):e10208.
11. Hovinga J.A., Vesely S.K., Terrell D.R., Lammle B., George J.N. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 2010, 115(8):1500-1511. [quiz 1662].
12. Bentley M.J., Lehman C.M., Blaylock R.C., Wilson A.R., Rodgers G.M. The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange. Transfusion 2010, 50(8):1654-1664.
13. Raife T., Atkinson B., Montgomery R., Vesely S., Friedman K. Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of thrombotic microangiopathy patients. Transfusion 2004, 44(2):146-150.
14. Patschan D., Witzke O., Duhrsen U., Erbel R., Philipp T., Herget-Rosenthal S. Acute myocardial infarction in thrombotic microangiopathies--clinical characteristics, risk factors and outcome. Nephrol Dial Transplant 2006, 21(6):1549-1554.
15. Hawkins B.M., Abu-Fadel M., Vesely S.K., George J.N. Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review. Transfusion 2008, 48(2):382-392.
16. Hughes C., McEwan J.R., Longair I., Hughes S., Cohen H., Machin S., et al. Cardiac involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13. J Thromb Haemost 2009, 7(4):529-536.
17. Hosler G.A., Cusumano A.M., Hutchins G.M. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. Arch Pathol Lab Med 2003, 127(7):834-839.
18. Asherson R.A., Cervera R. Microvascular and microangiopathic antiphospholipid-associated syndromes (" MAPS"): semantic or antisemantic?. Autoimmun Rev 2008, 7(3):164-167.
19. Schneppenheim R., Budde U., Oyen F., Angerhaus D., Aumann V., Drewke E., et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood 2003, 101(5):1845-1850.
20. Rock G.A., Shumak K.H., Buskard N.A., Blanchette V.S., Kelton J.G., Nair R.C., et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med 1991, 325(6):393-397.
21. Coppo P., Bussel A., Charrier S., Adrie C., Galicier L., Boulanger E., et al. High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome. Medicine (Baltimore) 2003, 82(1):27-38.
22. Pene F., Vigneau C., Auburtin M., Moreau D., Zahar J.R., Coste J., et al. Outcome of severe adult thrombotic microangiopathies in the intensive care unit. Intensive Care Med 2005, 31(1):71-78.
23. Rock G., Shumak K.H., Sutton D.M., Buskard N.A., Nair R.C. Cryosupernatant as replacement fluid for plasma exchange in thrombotic thrombocytopenic purpura. Members of the Canadian Apheresis Group. Br J Haematol 1996, 94(2):383-386.
24. Zeigler Z.R., Gryn J.F., Rintels P.B. Cryopoor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP). Blood 1998, 92(Suppl. 1):707a.
25. Bell W.R., Braine H.G., Ness P.M., Kickler T.S. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991, 325(6):398-403.
26. Balduini C.L., Gugliotta L., Luppi M., Laurenti L., Klersy C., Pieresca C., et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010, 89(6):591-596.
27. Rojnuckarin P., Watanaboonyongcharoen P., Akkawat B., Intragumtornchai T. The role of pulse dexamethasone in acquired idiopathic thrombotic thrombocytopenic purpura. J Thromb Haemost 2006, 4(5):1148-1150.
28. Toyoshige M., Zaitsu Y., Okafuji K., Inoue Y., Hiroshige Y., Matsumoto N., et al. Successful treatment of thrombotic thrombocytopenic purpura with high-dose corticosteroid. Am J Hematol 1992, 41(1):69.
29. Bobbio-Pallavicini E., Gugliotta L., Centurioni R., Porta C., Vianelli N., Billio A., et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 1997, 82(4):429-435.
30. Rosove M.H., Ho W.G., Goldfinger D. Ineffectiveness of aspirin and dipyridamole in the treatment of thrombotic thrombocytopenic purpura. Ann Intern Med 1982, 96(1):27-33.
31. Harkness D.R., Byrnes J.J., Lian E.C., Williams W.D., Hensley G.T. Hazard of platelet transfusion in thrombotic thrombocytopenic purpura. JAMA 1981, 246(17):1931-1933.
32. Gordon L.I., Kwaan H.C., Rossi E.C. Deleterious effects of platelet transfusions and recovery thrombocytosis in patients with thrombotic microangiopathy. Semin Hematol 1987, 24(3):194-201.
33. de la Rubia J., Plume G., Arriaga F., Carpio N., Sanz M.A., Marty M.L. Platelet transfusion and thrombotic thrombocytopenic purpura. Transfusion 2002, 42(10):1384-1385.
34. Tripathi S.P., Deshpande A.S., Khadse S., Kulkarni R.K. Case of TTP with cerebral infarct secondary to platelet transfusion. Indian J Pediatr 2011, 78(1):109-111.
35. Coppo P., Lassoued K., Mariette X., Gossot D., Oksenhendler E., Adrie C., et al. Effectiveness of platelet transfusions after plasma exchange in adult thrombotic thrombocytopenic purpura: a report of two cases. Am J Hematol 2001, 68(3):198-201.
36. Swisher K.K., Terrell D.R., Vesely S.K., Kremer Hovinga J.A., Lammle B., George J.N. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion 2009, 49(5):873-887.
37. Mannucci P.M., Bohm M., Scharrer I., Scheiflinger F. Patterns of changes of anti-ADAMTS13 after plasma exchange. J Thromb Haemost 2006, 4(6):1405-1406.
38. Caramazza D., Quintini G., Abbene I., Coco L.L., Malato A., Di Trapani R., et al. Rituximab for managing relapsing or refractory patients with idiopathic thrombotic thrombocytopenic purpura--haemolytic uraemic syndrome. Blood Transfus 2010, 8(3):203-210.
39. Scully M., McDonald V., Cavenagh J., Hunt B.J., Longair I., Cohen H., et al. A phase II study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood 2011, 118(7):1746-1753.
40. Froissart A., Buffet M., Veyradier A. First-line rituximab efficacy and safety in patients with acquired idiopathic thrombotic thrombocytopenic purpura experiencing a suboptimal response to therapeutical plasma exchange: results of a prospective multicenter phase 2 study. Crit Care Med 2012, 40(1):104-111.
41. Bobbio-Pallavicini E., Porta C., Centurioni R., Gugliotta L., Vianelli N., Tacconi F., et al. Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. The Italian Cooperative Group for TTP. Eur J Haematol 1994, 52(4):222-226.
42. Welborn J.L., Emrick P., Acevedo M. Rapid improvement of thrombotic thrombocytopenic purpura with vincristine and plasmapheresis. Am J Hematol 1990, 35(1):18-21.
43. Ziman A., Mitri M., Klapper E., Pepkowitz S.H., Goldfinger D. Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution's experience and review of the literature. Transfusion 2005, 45(1):41-49.
44. Onundarson P.T., Rowe J.M., Heal J.M., Francis C.W. Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura. A 10-year experience at a single institution. Arch Intern Med 1992, 152(4):791-796.
45. Kappers-Klunne M.C., Wijermans P., Fijnheer R., Croockewit A.J., van der Holt B., de Wolf J.T., et al. Splenectomy for the treatment of thrombotic thrombocytopenic purpura. Br J Haematol 2005, 130(5):768-776.
46. Zieschang M., Kohlhaufl M., Hoffler D., Niemeyer R., Prager G. A case of thrombotic thrombocytopenic purpura successfully treated with cyclophosphamide. Nephron 1995, 69(2):176.
47. Centurioni R., Bobbio-Pallavicini E., Porta C., Rodeghiero F., Gugliotta L., Billio A., et al. Treatment of thrombotic thrombocytopenic purpura with high-dose immunoglobulins. Results in 17 patients. Italian Cooperative Group for TTP. Haematologica 1995, 80(4):325-331.
48. Hand J.P., Lawlor E.R., Yong C.K., Davis J.H. Successful use of cyclosporine A in the treatment of refractory thrombotic thrombocytopenic purpura. Br J Haematol 1998, 100(3):597-599.
49. Nosari A., Bernuzzi P., Corneo R., Pungolino E., Muti G., Rossi V., et al. Late response to cyclosporine in refractory thrombotic thrombocytopenic purpura. Int J Hematol 2002, 76(3):284-286.
50. Itala M., Remes K. Excellent response of refractory life-threatening thrombotic thrombocytopenic purpura to cyclosporine treatment. Clin Lab Haematol 2004, 26(1):65-67.
51. Ahmad H.N., Thomas-Dewing R.R., Hunt B.J. Mycophenolate mofetil in a case of relapsed, refractory thrombotic thrombocytopenic purpura. Eur J Haematol 2007, 78(5):449-452.
52. Cataland S.R., Jin M., Lin S., Kennedy M.S., Kraut E.H., George J.N., et al. Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. Br J Haematol 2007, 139(3):486-493.
53. Cataland S.R., Jin M., Lin S., Kraut E.H., George J.N., Wu H.M. Effect of prophylactic cyclosporine therapy on ADAMTS13 biomarkers in patients with idiopathic thrombotic thrombocytopenic purpura. Am J Hematol 2008, 83(12):911-915.
54. Nguyen L., Li X., Duvall D., Terrell D.R., Vesely S.K., George J.N. Twice-daily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006. Transfusion 2008, 48(2):349-357.
55. Ferrari S., Scheiflinger F., Rieger M., Mudde G., Wolf M., Coppo P., et al. Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood 2007, 109(7):2815-2822.
56. Peyvandi F., Lavoretano S., Palla R., Feys H.B., Vanhoorelbeke K., Battaglioli T., et al. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica 2008, 93(2):232-239.
57. Lotta L.A., Mariani M., Consonni D., Mancini I., Palla R., Maino A., et al. Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura. Br J Haematol 2010, 151(5):488-494.
58. Veltman G.A., Brand A., Leeksma O.C., ten Bosch G.J., van Krieken J.H., Briet E. The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpura. Ann Hematol 1995, 70(5):231-236.
59. Kremer Hovinga J.A., Studt J.D., Demarmels Biasiutti F., Solenthaler M., Alberio L., Zwicky C., et al. Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura. Haematologica 2004, 89(3):320-324.
60. Moake J.L., Rudy C.K., Troll J.H., Schafer A.I., Weinstein M.J., Colannino N.M., et al. Therapy of chronic relapsing thrombotic thrombocytopenic purpura with prednisone and azathioprine. Am J Hematol 1985, 20(1):73-79.
61. Pasquale D., Vidhya R., DaSilva K., Tsan M.F., Lansing L., Chikkappa G. Chronic relapsing thrombotic thrombocytopenic purpura: role of therapy with cyclosporine. Am J Hematol 1998, 57(1):57-61.
62. Allan D.S., Kovacs M.J., Clark W.F. Frequently relapsing thrombotic thrombocytopenic purpura treated with cytotoxic immunosuppressive therapy. Haematologica 2001, 86(8):844-850.
63. Fujimura Y., Matsumoto M., Kokame K., Isonishi A., Soejima K., Akiyama N., et al. Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients. Br J Haematol 2009, 144(5):742-754.
64. Tao Z., Anthony K., Peng Y., Choi H., Nolasco L., Rice L., et al. Novel ADAMTS-13 mutations in an adult with delayed onset thrombotic thrombocytopenic purpura. J Thromb Haemost 2006, 4(9):1931-1935.
65. Camilleri R.S., Cohen H., Mackie I.J., Scully M., Starke R.D., Crawley J.T., et al. Prevalence of the ADAMTS-13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpura. J Thromb Haemost 2008, 6(2):331-338.
66. Malak S., Wolf M., Millot G.A., Mariotte E., Veyradier A., Meynard J.L., et al. Human immunodeficiency virus-associated thrombotic microangiopathies: clinical characteristics and outcome according to ADAMTS13 activity. Scand J Immunol 2008, 68(3):337-344.
67. Gunther K., Garizio D., Nesara P. ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura. Transfusion 2007, 47(9):1710-1716.
68. Novitzky N., Thomson J., Abrahams L., du Toit C., McDonald A. Thrombotic thrombocytopenic purpura in patients with retroviral infection is highly responsive to plasma infusion therapy. Br J Haematol 2005, 128(3):373-379.
69. Hart D., Sayer R., Miller R., Edwards S., Kelly A., Baglin T., et al. Human immunodeficiency virus associated thrombotic thrombocytopenic purpura--favourable outcome with plasma exchange and prompt initiation of highly active antiretroviral therapy. Br J Haematol 2011, 153(4):515-519.
70. Francis K.K., Kalyanam N., Terrell D.R., Vesely S.K., George J.N. Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: a report of 10 patients and a systematic review of published cases. Oncologist 2007, 12(1):11-19.
71. Oberic L., Buffet M., Schwarzinger M., Veyradier A., Clabault K., Malot S., et al. Cancer awareness in atypical thrombotic microangiopathies. Oncologist 2009, 14(8):769-779.
72. Zakarija A., Kwaan H.C., Moake J.L., Bandarenko N., Pandey D.K., McKoy J.M., et al. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). Kidney Int Suppl 2009, 112:S20-S24.
73. Scully M., Starke R., Lee R., Mackie I., Machin S., Cohen H. Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpura. Blood Coagul Fibrinolysis 2006, 17(6):459-463.
74. Horton T.M., Stone J.D., Yee D., Dreyer Z., Moake J.L., Mahoney D.H. Case series of thrombotic thrombocytopenic purpura in children and adolescents. J Pediatr Hematol Oncol 2003, 25(4):336-339.
75. Veyradier A., Obert B., Haddad E., Cloarec S., Nivet H., Foulard M., et al. Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome. J Pediatr 2003, 142(3):310-317.
76. Matsumoto M., Yagi H., Ishizashi H., Wada H., Fujimura Y. The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Semin Hematol 2004, 41(1):68-74.
77. Klukowska A., Niewiadomska E., Budde U., Oyen F., Schneppenheim R. Difficulties in diagnosing congenital thrombotic thrombocytopenic purpura. J Pediatr Hematol Oncol 2010, 32(2):103-107.
78. Bouw M.C., Dors N., van Ommen H., Ramakers-van Woerden N.L. Thrombotic thrombocytopenic purpura in childhood. Pediatr Blood Cancer 2009, 53(4):537-542.
79. Galbusera M., Noris M., Remuzzi G. Inherited thrombotic thrombocytopenic purpura. Haematologica 2009, 94(2):166-170.
80. Loirat C., Girma J.P., Desconclois C., Coppo P., Veyradier A. Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children. Pediatr Nephrol 2009, 24(1):19-29.
81. Albaramki J.H., Teo J., Alexander S.I. Rituximab therapy in two children with autoimmune thrombotic thrombocytopenic purpura. Pediatr Nephrol 2009, 24(9):1749-1752.
82. Sato A., Hoshi Y., Onuma M., Sato R., Tsunematsu Y., Isonishi A., et al. A 9-month-old infant with acquired idiopathic thrombotic thrombocytopenic purpura caused by inhibitory IgG-autoantibody to ADAMTS13. Pediatr Hematol Oncol 2010, 27(1):53-58.
83. Schneppenheim R., Budde U., Hassenpflug W., Obser T. Severe ADAMTS-13 deficiency in childhood. Semin Hematol 2004, 41(1):83-89.
84. Moskowitz N.P., Fligman I., Scimeca P., Weinblatt M. Transient TTP in childhood. Pediatr Blood Cancer 2009, 52(3):424-426.
85. McDonald V., Liesner R., Grainger J., Gattens M., Machin S.J., Scully M. Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assays. Blood Coagul Fibrinolysis 2010, 21(3):245-250.
86. Harambat J., Lamireau D., Delmas Y., Ryman A., Llanas B., Brissaud O. Successful treatment with rituximab for acute refractory thrombotic thrombocytopenic purpura related to acquired ADAMTS13 deficiency: a pediatric report and literature review. Pediatr Crit Care Med 2011, 12(2):e90-e93.
87. Muchitsch E.M., Dietrich B., Rottensteiner H., Auer W., Nehrbass D., Gritsch H., et al. Preclinical testing of human recombinant von Willebrand factor: ADAMTS13 cleavage capacity in animals as criterion for species suitability. Semin Thromb Hemost 2010, 36(5):522-528.
88. Plaimauer B., Kremer Hovinga J.A., Juno C., Wolfsegger M.J., Skalicky S., Schmidt M., et al. Recombinant ADAMTS13 normalizes von Willebrand factor-cleaving activity in plasma of acquired TTP patients by overriding inhibitory antibodies. J Thromb Haemost 2011, 9(5):936-944.
89. Jilma-Stohlawetz P., Gorczyca M.E., Jilma B., Siller-Matula J., Gilbert J.C., Knobl P. Inhibition of von Willebrand factor by ARC1779 in patients with acute thrombotic thrombocytopenic purpura. Thromb Haemost 2011, 105(3):545-552.
90. Chen J., Reheman A., Gushiken F.C., Nolasco L., Fu X., Moake J.L., et al. N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice. J Clin Invest 2011, 121(2):593-603.