Thrombotic thrombocytopenic purpura

References

Key articles

Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017 Feb;15(2):312-22.Full text  Abstract

Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35.Full text  Abstract

Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005 Jul;3(7):1432-6.Full text  Abstract

Moschowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89.

Moore JC, Hayward CP, Warkentin TE, et al. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98:1842-1846.Full text  Abstract

Banno F, Kokame K, Okuda T, et al. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006 Apr 15;107(8):3161-6.Full text  Abstract

Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost. 2005 Nov;3(11):2420-7. Abstract

Rock GA, Shumak KH, Buskard NA, 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 Aug 8;325(6):393-7. Abstract

Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021 Apr 8;137(14):1855-61.Full text  Abstract

Reference articles

1. Scully M, Cataland S, Coppo P, et al. Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost. 2017 Feb;15(2):312-22.Full text  Abstract

2. Vesely SK, George JN, Lammle B, et al. ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003;102:60-68.Full text  Abstract

3. Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020 Oct;18(10):2496-502.Full text  Abstract

4. Scully M, Hunt BJ, Benjamin S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012 Aug;158(3):323-35.Full text  Abstract

5. Tehrani HA, Darnahal M, Vaezi M, et al. COVID-19 associated thrombotic thrombocytopenic purpura (TTP); a case series and mini-review. Int Immunopharmacol. 2021 Apr;93:107397. Abstract

6. Altowyan E, Alnujeidi O, Alhujilan A, et al. COVID-19 presenting as thrombotic thrombocytopenic purpura (TTP). BMJ Case Rep. 2020 Dec 17;13(12):e238026.Full text  Abstract

7. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009;361:1676-1687. Abstract

8. Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006;108:1267-1279.Full text  Abstract

9. Yu J, Yuan X, Chen H, et al. Direct activation of the alternative complement pathway by SARS-CoV-2 spike proteins is blocked by factor D inhibition. Blood. 2020 Oct 29;136(18):2080-9.Full text  Abstract

10. Gavriilaki E, Brodsky RA. Severe COVID-19 infection and thrombotic microangiopathy: success does not come easily. Br J Haematol. 2020 Jun;189(6):e227-e230.Full text  Abstract

11. Chang JC, Kathula SK. Various clinical manifestations in patients with thrombotic microangiopathy. J Investig Med. 2002;50:201-206. Abstract

12. Wajima T, Johnson EH. Sudden cardiac death from TTP. Clin Appl Thromb Hemost. 2000;6:108-110. Abstract

13. Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017 May 25;129(21):2836-46.Full text  Abstract

14. Mariotte E, Azoulay E, Galicier L, et al. Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura): a cross-sectional analysis of the French national registry for thrombotic microangiopathy. Lancet Haematol. 2016 May;3(5):e237-45.Full text  Abstract

15. George JN, Al-Nouri ZL. Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. Hematology Am Soc Hematol Educ Program. 2012;2012:604-9.Full text  Abstract

16. Miller D, Kaye JA, Shea K, et al. Incidence of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Epidemiology. 2004;15:208-215. Abstract

17. Terrell DR, Williams LA, Vesely SK, et al. The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005 Jul;3(7):1432-6.Full text  Abstract

18. Sadler JE, Moake JL, Miyata T, et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2004;407-423.Full text  Abstract

19. Swart L, Schapkaitz E, Mahlangu JN. Thrombotic thrombocytopenic purpura: A 5-year tertiary care centre experience. J Clin Apher. 2019 Feb;34(1):44-50. Abstract

20. Moschowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries. Arch Intern Med. 1925;36:89.

21. Furlan M, Robles R, Lamie B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87:4223-4234.Full text  Abstract

22. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339:1585-1594. Abstract

23. Bianchi V, Robles R, Alberio L, et al. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002;100:710-713.Full text  Abstract

24. Moore JC, Hayward CP, Warkentin TE, et al. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98:1842-1846.Full text  Abstract

25. Banno F, Kokame K, Okuda T, et al. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006 Apr 15;107(8):3161-6.Full text  Abstract

26. Crawley JT, Scully MA. Thrombotic thrombocytopenic purpura: basic pathophysiology and therapeutic strategies. Hematology Am Soc Hematol Educ Program. 2013;2013:292-9. Abstract

27. Fujimura Y, Matsumoto M. Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008. Intern Med. 2010;49(1):7-15.Full text  Abstract

28. Blake-Haskins JA, Lechleider RJ, Kreitman RJ. Thrombotic microangiopathy with targeted cancer agents. Clin Cancer Res. 2011 Sep 15;17(18):5858-66. Abstract

29. Sadler JE, Moake JL, Miyata T, et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2004:407-23.Full text  Abstract

30. Nicol KK, Shelton BJ, Knovich MA, et al. Overweight individuals are at increased risk for thrombotic thrombocytopenic purpura. Am J Hematol. 2003 Nov;74(3):170-4.Full text  Abstract

31. McMinn JR, George JN. Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. J Clin Apher. 2001;16(4):202-9. Abstract

32. Lesesne JB, Rothschild N, Erickson B, et al. Cancer-associated hemolytic-uremic syndrome: analysis of 85 cases from a national registry. J Clin Oncol. 1989 Jun;7(6):781-9. Abstract

33. Page Y, Tardy B, Zeni F, et al. Thrombotic thrombocytopenic purpura related to ticlopidine. Lancet. 1991 Mar 30;337(8744):774-6. Abstract

34. Dzik WH, Georgi BA, Khettry U, et al. Cyclosporine-associated thrombotic thrombocytopenic purpura following liver transplantation - successful treatment with plasma exchange. Transplantation. 1987 Oct;44(4):570-2. Abstract

35. Meisenberg BR, Robinson WL, Mosley CA, et al. Thrombotic thrombocytopenic purpura in human immunodeficiency (HIV)-seropositive males. Am J Hematol. 1988 Mar;27(3):212-5. Abstract

36. Daly AS, Xenocostas A, Lipton JH. Transplantation-associated thrombotic microangiopathy: twenty-two years later. Bone Marrow Transplant. 2002 Dec;30(11):709-15.Full text  Abstract

37. Bennett CL, Connors JM, Carwile JM, et al. Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med. 2000 Jun 15;342(24):1773-7. Abstract

38. Zakarija A, Kwaan HC, Moake JL, et al. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). Kidney Int Suppl. 2009 Feb;(112):S20-4. Abstract

39. Kojouri K, Vesely S, George JN. Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes. Ann Intern Med. 2001 Dec 18;135(12):1047-51. Abstract

40. Patton JF, Manning KR, Case D, et al. Serum lactate dehydrogenase and platelet count predict survival in thrombotic thrombocytopenic purpura. Am J Hematol. 1994 Oct;47(2):94-9. Abstract

41. Masias C, Cataland SR. The role of ADAMTS13 testing in the diagnosis and management of thrombotic microangiopathies and thrombosis. Blood. 2018 Aug 30;132(9):903-10.Full text  Abstract

42. Hubbard AR, Heath AB, Kremer Hovinga JA, et al. Establishment of the WHO 1st International Standard ADAMTS13, plasma (12/252): communication from the SSC of the ISTH. J Thromb Haemost. 2015 Jun;13(6):1151-3.Full text  Abstract

43. Zheng XL, Wu HM, Shang D, et al. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura. Haematologica. 2010 Sep;95(9):1555-62.Full text  Abstract

44. Veyradier A, Meyer D. Thrombotic thrombocytopenic purpura and its diagnosis. J Thromb Haemost. 2005 Nov;3(11):2420-7. Abstract

45. Shih AW, McFarlane A, Verhovsek M. Haptoglobin testing in hemolysis: measurement and interpretation. Am J Hematol. 2014 Apr;89(4):443-7. Abstract

46. Vesely SK, George JN, Lammle B, et al. ADAMTS-13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003 Jul 1;102(1):60-8.Full text  Abstract

47. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009 Oct 22;361(17):1676-87. Abstract

48. Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006 Aug 15;108(4):1267-79.Full text  Abstract

49. Francis KK, Kalyanam N, Terrell DR, et al. Disseminated malignancy misdiagnosed as thrombotic thrombocytopenic purpura: a report of 10 patients and a systematic review of published cases. Oncologist. 2007 Jan;12(1):11-9.Full text  Abstract

50. Booth KK, Terrell DR, Vesely SK, et al. Systemic infections mimicking thrombotic thrombocytopenic purpura. Am J Hematol. 2011 Sep;86(9):743-51. Abstract

51. Dold S, Singh R, Sarwar H, et al. Frequency of microangiopathic hemolytic anemia in patients with systemic lupus erythematosus exacerbation: distinction from thrombotic thrombocytopenic purpura, prognosis, and outcome. Arthritis Rheum. 2005 Dec 15;53(6):982-5.Full text  Abstract

52. George JN. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med. 2006 May 4;354(18):1927-35. Abstract

53. Pereira A, Mazzara R, Monteagudo J, et al. Thrombotic thrombocytopenic purpura⁄hemolytic uremic syndrome: a multivariate analysis of factors predicting the response to plasma exchange. Ann Hematol. 1995 Jun;70(6):319-23. Abstract

54. Michael M, Elliott EJ, Craig JC, et al. Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials. Am J Kidney Dis. 2009 Feb;53(2):259-72. Abstract

55. Michael M, Elliott EJ, Ridley GF, et al. Interventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Cochrane Database Syst Rev. 2009 Jan 21;(1):CD003595.Full text  Abstract

56. Brunskill SJ, Tusold A, Benjamin S, et al. A systematic review of randomized controlled trials for plasma exchange in the treatment of thrombotic thrombocytopenic purpura. Transfus Med. 2007 Feb;17(1):17-35. Abstract

57. Qu L, Kiss JE. Thrombotic microangiopathy in transplantation and malignancy. Semin Thromb Hemost. 2005 Dec;31(6):691-9. Abstract

58. Rock GA, Shumak KH, Buskard NA, 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 Aug 8;325(6):393-7. Abstract

59. Bell WR, Braine HG, Ness PM, et al. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. 1991 Aug 8;325(6):398-403.Full text  Abstract

60. Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019 Jan 9;380(4):335-46. Abstract

61. Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021 Apr 8;137(14):1855-61.Full text  Abstract

62. Swisher KK, Terrell DR, Vesely SK, et al. Clinical outcomes after platelet transfusions in patients with thrombotic thrombocytopenic purpura. Transfusion. 2009 May;49(5):873-87. Abstract

63. Elliott MA, Heit JA, Pruthi RK, et al. Rituximab for refractory and or relapsing thrombotic thrombocytopenic purpura related to immune-mediated severe ADAMTS13-deficiency: a report of four cases and a systematic review of the literature. Eur J Haematol. 2009 Oct;83(4):365-72. Abstract

64. Page EE, Kremer Hovinga JA, Terrell DR, et al. Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2016 Jun 16;127(24):3092-4.Full text  Abstract

65. Owattanapanich W, Wongprasert C, Rotchanapanya W, et al. Comparison of the long-term remission of rituximab and conventional treatment for acquired thrombotic thrombocytopenic purpura: a systematic review and meta-analysis. Clin Appl Thromb Hemost. 2019 Jan-Dec;25:1076029618825309.Full text  Abstract

66. Bobbio-Pallavivini E, Gugliotta L, Centurioni R, et al. Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica. 1997 Jul-Aug;82(4):429-35.Full text  Abstract

67. Welborn JL, Emrick P, Acevedo M. Rapid improvement of thrombotic thrombocytopenic purpura with vincristine and plasmapheresis. Am J Hematol. 1990 Sep;35(1):18-21. Abstract

68. O'Connor NT, Bruce-Jones P, Hill LF. Vincristine therapy for thrombotic thrombocytopenic purpura. Am J Hematol. 1992 Mar;39(3):234-6. Abstract

69. Bird JM, Cummins D, Machin SJ. Cyclophosphamide for chronic relapsing thrombotic thrombocytopenic purpura. Lancet. 1990 Sep 1;336(8714):565-6. Abstract

70. Udvardy M, Rak K. Cyclophosphamide for chronic relapsing thrombotic thrombocytopenic purpura. Lancet. 1990 Dec 15;336(8729):1508-9. Abstract

71. Strutz F, Wieneke U, Braess J, et al. Treatment of relapsing thrombotic thrombocytopenic purpura with cyclophosphamide pulse therapy. Nephrol Dial Transplant. 1998 May;13(5):1320-1.Full text  Abstract

72. Pasquale D, Vidhya R, DaSilva K, et al. Chronic relapsing thrombotic thrombocytopenic purpura: role of therapy with cyclosporine. Am J Hematol. 1998 Jan;57(1):57-61.Full text  Abstract

73. van Ojik H, Biesma DW, Fijnheer R, et al. Cyclosporine for thrombotic thrombocytopenic purpura after autologous bone marrow transplantation. Br J Haematol. 1997 Mar;96(3):641-3. Abstract

74. Outshoorn UM, Ferber A. Outcomes in the treatment of thrombotic thrombocytopenic purpura with splenectomy: a retrospective cohort study. Am J Hematol. 2006 Dec;81(12):895-900. Abstract

75. Dubois L, Gray DK. Case series: splenectomy: does it still play a role in the management of thrombotic thrombocytopenic purpura? Can J Surg. 2010 Oct;53(5):349-55.Full text  Abstract

76. 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. Transfusion. 2002;42:572-580. Abstract

77. Zheng XL, Kaufman RM, Goodnough LT, et al. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood. 2004;103:4043-4049.Full text  Abstract

78. Coppo P, Wolf M, Veyradier A, et al. Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol. 2006;132:66. Abstract

79. Patschan D, Witzke O, Durhsen U, et al. Acute myocardial infarction in thrombotic microangiopathies - clinical characteristics, risk factors, and outcome. Nephrol Dial Transplant. 2006 Jun;21(6):1549-54.Full text  Abstract

80. Howard MA, Williams LA, Terrell DR, et al. Complications of plasma exchange in patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Transfusion. 2006 Jan;46(1):154-6. Abstract

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