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Intensive Care Medicine
Published in: Intensive Care Medicine 11/2019

01-11-2019 | Thrombotic Thrombocytopenic Purpura | Seven-Day Profile Publication

Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura

Authors: Elie Azoulay, Philippe R. Bauer, Eric Mariotte, Lene Russell, Paul Knoebl, Ignacio Martin-Loeches, Frédéric Pène, Kathryn Puxty, Pedro Povoa, Andreas Barratt-Due, Jose Garnacho-Montero, Julia Wendon, Laveena Munshi, Dominique Benoit, Michael von Bergwelt-Baildon, Marco Maggiorini, Paul Coppo, Spero Cataland, Agnès Veyradier, Andry Van de Louw, On behalf of the Nine-i Investigators

Published in: Intensive Care Medicine | Issue 11/2019

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Abstract

Thrombotic thrombocytopenic purpura (TTP) is fatal in 90% of patients if left untreated and must be diagnosed early to optimize patient outcomes. However, the very low incidence of TTP is an obstacle to the development of evidence-based clinical practice recommendations, and the very wide variability in survival rates across centers may be partly ascribable to differences in management strategies due to insufficient guidance. We therefore developed an expert statement to provide trustworthy guidance about the management of critically ill patients with TTP. As strong evidence was difficult to find in the literature, consensus building among experts could not be reported for most of the items. This expert statement is timely given the recent advances in the treatment of TTP, such as the use of rituximab and of the recently licensed drug caplacizumab, whose benefits will be maximized if the other components of the management strategy follow a standardized pattern. Finally, unanswered questions are identified as topics of future research on TTP.
Literature
1.
Rock GA, Shumak KH, Buskard NA, Canadian Apheresis Study Group et al (1991) Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. N Engl J Med 325:393–397PubMed
2.
Bell WR, Braine HG, Ness PM, Kickler TS (1991) Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients. N Engl J Med 325:398–403PubMed
3.
Dong J, Moake JL, Nolasco L et al (2002) ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 100:4033–4039PubMed
4.
Veyradier A, Obert B, Houllier A, Meyer D, Girma JP (2001) Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood 98:1765–1772PubMed
5.
Levy GG, Nichols WC, Lian EC et al (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 413:488–494PubMed
6.
Tsai HM, Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339:1585–1594PubMedPubMedCentral
7.
Furlan M, Robles R, Galbusera M et al (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339:1578–1584PubMed
8.
Mannucci PM (2015) Understanding organ dysfunction in thrombotic thrombocytopenic purpura. Intensive Care Med 41:715–718PubMed
9.
Mariotte E, Azoulay E, Galicier L et al (2016) 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 3:e237–e245PubMed
10.
Scully M, Hunt BJ, Benjamin S et al (2012) Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 158:323–335PubMed
11.
Peyvandi F, Palla R, Lotta LA, Mackie I, Scully MA, Machin SJ (2010) ADAMTS-13 assays in thrombotic thrombocytopenic purpura. J Thromb Haemost JTH 8:631–640PubMed
12.
Joly BS, Coppo P, Veyradier A (2017) Thrombotic thrombocytopenic purpura. Blood 129:2836–2846PubMed
13.
Grall M, Azoulay E, Galicier L et al (2017) Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: causes of diagnostic errors and consequence on outcome. Experience of the French thrombotic microangiopathies reference centre. Am J Hematol 92:381–387PubMed
14.
Peigne V, Perez P, Resche Rigon M et al (2012) Causes and risk factors of death in patients with thrombotic microangiopathies. Intensive Care Med 38:1810–1817PubMed
15.
Pene F, Vigneau C, Auburtin M et al (2005) Outcome of severe adult thrombotic microangiopathies in the intensive care unit. Intensive Care Med 31:71–78PubMed
16.
Darmon M, Azoulay E, Thiery G et al (2006) Time course of organ dysfunction in thrombotic microangiopathy patients receiving either plasma perfusion or plasma exchange. Crit Care Med 34:2127–2133PubMed
17.
Scully M, Cataland SR, Peyvandi F et al (2019) Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med 380:335–346PubMed
18.
Fourmont AM, Zafrani L, Mariotte E et al (2018) The clinical features of cardiac involvement in patients with severe thrombotic thrombocytopenic purpura. Intensive Care Med 88:8–10
19.
Coppo P (2017) French reference center for thrombotic microangiopathies. Management of thrombotic thrombocytopenic purpura. Transfus Clin Biol J Soc Francaise Transfus Sang 24:148–153
20.
Matsumoto M, Fujimura Y, Wada H et al (2017) Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan. Int J Hematol 106:3–15PubMed
21.
Sills RH (1984) Thrombotic thrombocytopenic purpura. II. Principles of therapy and guidelines for management. Am J Pediatr Hematol Oncol 6:431–439PubMed
22.
Guyatt GH, Oxman AD, Vist GE et al (2008) GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 336:924–926PubMedPubMedCentral
23.
Atkins D, Best D, Briss PA et al (2004) Grading quality of evidence and strength of recommendations. BMJ 328:1490PubMed
24.
Zafrani L, Mariotte E, Darmon M et al (2015) Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS13 activity. J Thromb Haemost JTH 13:380–389PubMed
25.
Mariotte E, Blet A, Galicier L et al (2013) Unresponsive thrombotic thrombocytopenic purpura in critically ill adults. Intensive Care Med 39:1272–1281PubMed
26.
Knöbl P, Rintelen C, Kornek G et al (1997) Plasma exchange for treatment of thrombotic thrombocytopenic purpura in critically ill patients. Intensive Care Med 23:44–50PubMed
27.
Gasparović V, Radonić R, Mejić S, Pisl Z, Radman I (2000) Possibilities and limits of treatment in patients with thrombotic thrombocytopenic purpura. Intensive Care Med 26:1690–1693PubMed
28.
Burrus TM, Mandrekar J, Wijdicks EFM, Rabinstein AA (2010) Renal failure and posterior reversible encephalopathy syndrome in patients with thrombotic thrombocytopenic purpura. Arch Neurol 67:831–834PubMed
29.
Burrus TM, Wijdicks EFM, Rabinstein AA (2009) Brain lesions are most often reversible in acute thrombotic thrombocytopenic purpura. Neurology 73:66–70PubMed
30.
Benhamou Y, Boelle P-Y, Baudin B et al (2015) Cardiac troponin-I on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura. Experience of the French Thrombotic Microangiopathies Reference Center. J Thromb Haemost JTH 13:293–302PubMed
31.
McDonald V, Laffan M, Benjamin S, Bevan D, Machin S, Scully MA (2009) Thrombotic thrombocytopenic purpura precipitated by acute pancreatitis: a report of seven cases from a regional UK TTP registry. Br J Haematol 144:430–433PubMed
32.
Azoulay E, Knoebl P, Garnacho-Montero J et al (2017) Expert statements on the standard of care in critically ill adult patients with atypical hemolytic uremic syndrome. Chest 152:424–434PubMed
33.
Booth KK, Terrell DR, Vesely SK, George JN (2011) Systemic infections mimicking thrombotic thrombocytopenic purpura. Am J Hematol 86:743–751PubMedPubMedCentral
34.
Shatzel JJ, Taylor JA (2017) Syndromes of thrombotic microangiopathy. Med Clin North Am 101:395–415PubMed
35.
McClain RS, Terrell DR, Vesely SK, George JN (2014) Plasma exchange complications in patients treated for thrombotic thrombocytopenia purpura-hemolytic uremic syndrome: 2011–2014. Transfusion (Paris) 54:3257–3259
36.
Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN (2017) Thrombotic thrombocytopenic purpura: diagnostic criteria, clinical features, and long-term outcomes from 1995 through 2015. Blood Adv 1:590–600PubMedPubMedCentral
37.
Amorosi E, Ultmann J (1966) Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore). 45:139–159
38.
Coppo P, Schwarzinger M, Buffet M et al (2010) Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One 5:e10208PubMedPubMedCentral
39.
Bentley MJ, Lehman CM, Blaylock RC, Wilson AR, Rodgers GM (2010) The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange. Transfusion (Paris) 50:1654–1664
40.
Bentley MJ, Wilson AR, Rodgers GM (2013) Performance of a clinical prediction score for thrombotic thrombocytopenic purpura in an independent cohort. Vox Sang 105:313–318PubMed
41.
Bendapudi PK, Hurwitz S, Fry A et al (2017) Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 4:e157–e164PubMed
42.
Li A, Khalighi PR, Wu Q, Garcia DA (2018) External validation of the PLASMIC score: a clinical prediction tool for thrombotic thrombocytopenic purpura diagnosis and treatment. J Thromb Haemost JTH 16:164–169PubMed
43.
Kaplan AA (2013) Therapeutic plasma exchange: a technical and operational review: therapeutic plasma exchange. J Clin Apher 28:3–10PubMed
44.
Reeves HM, Winters JL (2014) The mechanisms of action of plasma exchange. Br J Haematol 164:342–351PubMed
45.
Wu N, Liu J, Yang S et al (2015) Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura: diagnostic and prognostic values of ADAMTS13 during PE Therapy. Transfusion (Paris) 55:18–24
46.
Scully M, Yarranton H, Liesner R et al (2008) Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical features. Br J Haematol 142:819–826PubMed
47.
Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN (2010) Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood 115:1500–1511PubMed
48.
Benhamou Y, Boelle PY, Baudin B et al (2015) Cardiac troponin-i on diagnosis predicts early death and refractoriness in acquired thrombotic thrombocytopenic purpura: Experience of the French Thrombotic Microangiopathies Reference Center. J Thromb Haemost 13:293–302PubMed
49.
Hawkins BM, Abu-Fadel M, Vesely SK, George JN (2008) Clinical cardiac involvement in thrombotic thrombocytopenic purpura: a systematic review. Transfusion (Paris) 48:382–392
50.
Hosler GA, Cusumano AM, Hutchins GM (2003) Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities: a review of 56 autopsy cases. Arch Pathol Lab Med 127:834–839PubMed
51.
Shah N, Rutherford C, Matevosyan K, Shen Y-M, Sarode R (2013) Role of ADAMTS13 in the management of thrombotic microangiopathies including thrombotic thrombocytopenic purpura (TTP). Br J Haematol 163:514–519PubMed
52.
Vesely SK, George JN, Lämmle B et al (2003) ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 102:60–68PubMed
53.
Lotta LA, Mariani M, Consonni D et al (2010) Different clinical severity of first episodes and recurrences of thrombotic thrombocytopenic purpura. Br J Haematol 151:488–494PubMed
54.
Benhamou Y, Baudel J-L, Wynckel A et al (2015) Are platelet transfusions harmful in acquired thrombotic thrombocytopenic purpura at the acute phase? Experience of the French Thrombotic Microangiopathies Reference Center. Am J Hematol 90:127–129
55.
Yoshii Y, Fujimura Y, Bennett CL, Isonishi A, Kurumatani N, Matsumoto M (2017) Implementation of a rapid assay of ADAMTS13 activity was associated with improved 30-day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet transfusions. Transfusion (Paris) 57:2045–2053
56.
Mörtzell M, Berlin G, Nilsson T et al (2011) Analyses of data of patients with Thrombotic Microangiopathy in the WAA registry. Transfus Apher Sci 45:125–131PubMed
57.
Cataland SR, Yang S, Witkoff L et al (2009) Demographic and ADAMTS13 biomarker data as predictors of early recurrences of idiopathic thrombotic thrombocytopenic purpura. Eur J Haematol 83:559–564PubMed
58.
Von Veyradier A (2016) Willebrand factor–a new target for TTP treatment? N Engl J Med 374:583–585
59.
Rose M, Eldor A (1987) High incidence of relapses in thrombotic thrombocytopenic purpura. Clinical study of 38 patients. Am J Med 83:437–444PubMed
60.
Levandovsky M, Harvey D, Lara P, Wun T (2008) Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients. J Hematol OncolJ Hematol Oncol 1:23
61.
Ridolfi RL, Hutchins GM, Bell WR (1979) The heart and cardiac conduction system in thrombotic thrombocytopenic purpura. A clinicopathologic study of 17 autopsied patients. Ann Intern Med 91:357–363PubMed
62.
Sane DC, Streer NP, Owen J (2009) Myocardial necrosis in patients with thrombotic thrombocytopenic purpura: pathophysiology and rationale for specific therapy. Eur J Haematol 82:83–92PubMed
63.
Brazelton J, Oster RA, McCleskey B, Fuller J, Adamski J, Marques MB (2017) Increased troponin I is associated with fatal outcome in acquired thrombotic thrombocytopenic purpura. J Clin Apheresis 32:311–318PubMed
64.
Benhamou Y, Assié C, Boelle PY et al (2012) Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. Haematologica 97:1181–1186PubMedPubMedCentral
65.
Berti-de-Marinis G, Novello S, Ferrari S et al (2016) Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients. J Thromb Thrombolysis 42:586–592PubMed
66.
Goel R, King KE, Takemoto CM, Ness PM, Tobian AAR (2016) Prognostic risk-stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion (Paris) 56:1451–1458
67.
Chaturvedi S, Carcioppolo D, Zhang L, Mccrae KR (2013) Management and outcomes for patients with TTP: analysis of 100 cases at a single institution. Am J Hematol 88:560–565PubMed
68.
Scully M, Cataland S, Coppo P et al (2017) Consensus on the standardization of terminology in thrombotic thrombocytopenic purpura and related thrombotic microangiopathies. J Thromb Haemost JTH 15:312–322PubMed
69.
Coppo P, Froissart A, French Reference Center for Thrombotic Microangiopathies (2015) Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange. Hematol Am Soc Hematol Educ Program 2015:637–643
70.
Moatti-Cohen M, Garrec C, Wolf M et al (2012) Unexpected frequency of Upshaw-Schulman syndrome in pregnancy-onset thrombotic thrombocytopenic purpura. Blood 119:5888–5897PubMed
71.
Scully M, Thomas M, Underwood M et al (2014) Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomes. Blood 124:211–219PubMed
72.
Uriol Rivera MG, Cabello Pelegrin S, Ballester Ruiz C et al (2018) Impact of a multidisciplinary team for the management of thrombotic microangiopathy. PLoS One 13:e0206558PubMedPubMedCentral
73.
Moake J (2009) Thrombotic thrombocytopenia purpura (TTP) and other thrombotic microangiopathies. Best Pract Res Clin Haematol 22:567–576PubMed
74.
Coppo P, Bussel A, Charrier S et al (2003) High-dose plasma infusion versus plasma exchange as early treatment of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome. Medicine (Baltimore) 82:27–38
75.
Balduini CL, Gugliotta L, Luppi M et al (2010) High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 89:591–596PubMed
76.
Becerra E, Scully MA, Leandro MJ et al (2015) Effect of rituximab on B cell phenotype and serum B cell-activating factor levels in patients with thrombotic thrombocytopenic purpura. Clin Exp Immunol 179:414–425PubMedPubMedCentral
77.
Benhamou Y, Paintaud G, Azoulay E et al (2016) Efficacy of a rituximab regimen based on B cell depletion in thrombotic thrombocytopenic purpura with suboptimal response to standard treatment: results of a phase II, multicenter noncomparative study. Am J Hematol 91:1246–1251PubMed
78.
Fakhouri F, Vernant J-P, Veyradier A et al (2005) Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood 106:1932–1937PubMed
79.
Fakhouri F, Teixeira L, Delarue R, Grünfeld J-P, Veyradier A (2004) Responsiveness of thrombotic thrombocytopenic purpura to rituximab and cyclophosphamide. Ann Intern Med 140:314–315PubMed
80.
Froissart A, Buffet M, Veyradier A et al (2012) Efficacy and safety of first-line rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med 40:104–111PubMed
81.
Page EE, Kremer Hovinga JA, Terrell DR, Vesely SK, George JN (2016) Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura. Blood 127:3092–3094PubMed
82.
Scully M, McDonald V, Cavenagh J et al (2011) A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood 118:1746–1753PubMed
83.
Westwood J-P, Webster H, McGuckin S, McDonald V, Machin SJ, Scully M (2013) Rituximab for thrombotic thrombocytopenic purpura: benefit of early administration during acute episodes and use of prophylaxis to prevent relapse. J Thromb Haemost JTH 11:481–490PubMed
84.
Scully M, Cohen H, Cavenagh J et al (2007) Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol 136:451–461PubMed
85.
Cataland SR, Wu HM (2005) Immunotherapy for thrombotic thrombocytopenic purpura. Curr Opin Hematol 12:359–363PubMed
86.
Cataland SR, Jin M, Ferketich AK et al (2007) An evaluation of cyclosporin and corticosteroids individually as adjuncts to plasma exchange in the treatment of thrombotic thrombocytopenic purpura. Br J Haematol 136:146–149PubMed
87.
Cataland SR, Jin M, Lin S et al (2007) Cyclosporin and plasma exchange in thrombotic thrombocytopenic purpura: long-term follow-up with serial analysis of ADAMTS13 activity. Br J Haematol 139:486–493PubMed
88.
Beloncle F, Buffet M, Coindre J-P et al (2012) Splenectomy and/or cyclophosphamide as salvage therapies in thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. Transfusion (Paris) 52:2436–2444
89.
Durand JM, Lefevre P, Kaplanski G, Telle H, Soubeyrand J (1992) Vincristine for thrombotic thrombocytopenic purpura. Lancet Lond Engl 340:977–978
90.
91.
Thomas MR, Robinson S, Scully MA (2016) How we manage thrombotic microangiopathies in pregnancy. Br J Haematol 173:821–830PubMed
92.
Shortt J, Oh DH, Opat SS (2013) ADAMTS13 antibody depletion by bortezomib in thrombotic thrombocytopenic purpura. N Engl J Med 368:90–92PubMed
93.
94.
Cabanillas G, Popescu-Martinez A (2016) N-acetylcysteine for relapsing thrombotic thrombocytopenic purpura: more evidence of a promising drug. Am J Ther 23:e1277–e1279PubMed
95.
Peyvandi F, Scully M, Kremer Hovinga JA et al (2017) Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost JTH 15:1448–1452PubMed
96.
Knoebl P, Scully M, Cataland S et al (2018) Integrated safety results from the phase II and phase III studies with caplacizumab in patients with acquired thrombotic thrombocytopenic purpura. Blood 132:3739
97.
Cataland SR, Scully M, Peyvandi F et al (2018) Risk factors and manageability of the mainly mild mucocutaneous bleeding profile observed in aTTP patients treated with caplacizumab during the phase III hercules study. Blood 132:1142
98.
Riviere E, Saint-Léger M, James C et al (2015) Platelet transfusion and catheter insertion for plasma exchange in patients with thrombotic thrombocytopenic purpura and a low platelet count. Transfusion (Paris) 55:1798–1802
99.
Hellstern P, Muntean W, Schramm W, Seifried E, Solheim BG (2002) Practical guidelines for the clinical use of plasma. Thromb Res 107(Suppl 1):S53–S57PubMed
100.
Coppo P, French Reference Center for Thrombotic Microangiopathies (2017) Treatment of autoimmune thrombotic thrombocytopenic purpura in the more severe forms. Transfus Apher Sci 56:52–56PubMed
101.
Som S, Deford CC, Kaiser ML et al (2012) Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996–2011. Transfusion (Paris) 52:2525–2532
102.
Vazquez-Mellado A, Pequeño-Luévano M, Cantu-Rodriguez OG et al (2016) More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura. Hematol Amst Neth 21:311–316
103.
Peyvandi F, Scully M, Kremer Hovinga JA et al (2016) Caplacizumab for acquired thrombotic thrombocytopenic purpura. N Engl J Med 374:511–522PubMed
104.
Bobbio-Pallavicini E, Gugliotta L, Centurioni R et al (1997) Antiplatelet agents in thrombotic thrombocytopenic purpura (TTP). Results of a randomized multicenter trial by the Italian Cooperative Group for TTP. Haematologica 82:429–435PubMed
105.
Hie M, Gay J, Galicier L et al (2014) Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura. Blood 124:204–210PubMed
106.
Coppo P, Wolf M, Veyradier A et al (2006) Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol 132:66–74PubMed
107.
van der Veen BS, Besseling R, Hoogendoorn M (2015) Serial ADAMTS13 measurements during initial plasma exchange therapy guide decisions for management of unresponsive thrombotic thrombocytopenic purpura. Transfusion (Paris) 55:2511–2515
108.
Jestin M, Benhamou Y, Schelpe A-S et al (2018) Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura. Blood 132:2143–2153PubMed
109.
Cataland SR, Scully MA, Paskavitz J et al (2011) Evidence of persistent neurologic injury following thrombotic thrombocytopenic purpura. Am J Hematol 86:87–89PubMed
110.
Soucemarianadin M, Benhamou Y, Delmas Y et al (2016) Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience. Eur J Haematol 97:183–191PubMed
111.
Kahwash E, Lockwood WB (2004) Twice daily plasma exchange in refractory thrombotic thrombocytopenic purpura. Ther Apher Dial 8:254–257PubMed
112.
Delgado-Lamas JL, Romero-García F, Rodriguez-Carrillo J (1991) Treatment of thrombotic thrombocytopenic purpura using vincristine and factor VIII plasma free. Am J Hematol 38:77PubMed
113.
Zheng X, Pallera AM, Goodnough LT, Sadler JE, Blinder MA (2003) Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab. Ann Intern Med 138:105–108PubMed
114.
Al-Samkari H, Grace RF, Connors JM (2018) Ofatumumab for acute treatment and prophylaxis of a patient with multiple relapses of acquired thrombotic thrombocytopenic purpura. J Thromb Thrombolysis 46:81–83PubMed
115.
Modic M, Cernelc P, Zver S (2002) Splenectomy: the last option of immunosuppressive therapy in patients with chronic or relapsing idiopathic thrombotic thrombocytopenic purpura? Transpl Proc 34:2953–2954
116.
Schwartz J, Eldor A, Szold A (2001) Laparoscopic splenectomy in patients with refractory or relapsing thrombotic thrombocytopenic purpura. Arch Surg Chic Ill 136:1236–1238
117.
Wu N, Liu J, Yang S et al (2015) Diagnostic and prognostic values of ADAMTS13 activity measured during daily plasma exchange therapy in patients with acquired thrombotic thrombocytopenic purpura: diagnostic and prognostic values of ADAMTS13 during PE therapy. Transfusion 55:18–24. https://​doi.​org/​10.​1111/​trf.​12762 CrossRefPubMed
118.
119.
120.
121.
122.
123.
124.
125.
126.
127.
128.
129.
130.
131.
132.
133.
Bobbio-Pallavicini E, Porta C, Centurioni R et al (1994) Vincristine sulfate for the treatment of thrombotic thrombocytopenic purpura refractory to plasma-exchange. The Italian Cooperative Group for TTP. Eur J Haematol 52:222–226PubMed
Metadata
Title
Expert statement on the ICU management of patients with thrombotic thrombocytopenic purpura
Authors
Elie Azoulay
Philippe R. Bauer
Eric Mariotte
Lene Russell
Paul Knoebl
Ignacio Martin-Loeches
Frédéric Pène
Kathryn Puxty
Pedro Povoa
Andreas Barratt-Due
Jose Garnacho-Montero
Julia Wendon
Laveena Munshi
Dominique Benoit
Michael von Bergwelt-Baildon
Marco Maggiorini
Paul Coppo
Spero Cataland
Agnès Veyradier
Andry Van de Louw
On behalf of the Nine-i Investigators
Publication date
01-11-2019
Publisher
Springer Berlin Heidelberg
Published in
Intensive Care Medicine / Issue 11/2019
Print ISSN: 0342-4642
Electronic ISSN: 1432-1238
DOI
https://doi.org/10.1007/s00134-019-05736-5

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